Dementia Treatment

Super Memory Formula

After the harsh reality that the doctor had to face his son ending his life, he suffered a major irreversible memory loss disease. This caused him to fall into depression and depend on the drugs from the pharma which was devasting for his mental and physical health and on so many other levels. After countless hours of research and experimentation, he realized that the root of all problems of memory loss was an enzyme that eats away the memory cells when the person gets older. This makes the person forget their loved ones, family and friends as if they have never met them. In some cases, they even forget about their past experiences, if they had children, how they came to the place they are in right now and who they are in the first place. This was exactly what the doctor had in his future if he did not make a decision. But he did and met with great people who helped him find the cure. This was a groundbreaking study that no one wanted to believe or endorse because it would go against the large pharma industry. However, the information is in there to protect yourself and your loved ones from such a devastating experience. You only need to follow the link and you will be guided to get the information downloaded to your device and follow the all-natural ways to get rid of memory loss. More here...

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Degenerative diseases and dementia

The most common neurodegenerative disorders are the dementias in late life. Six per cent of persons over the age of 65 years have dementia, and the rate increases exponentially as a function of age. Alzheimer's disease is the most common cause of dementia, and patients with Alzheimer's disease are six times more likely to develop epilepsy than age-matched controls. Partial and secondarily generalized seizures occur, and are usually relatively easily treated with conventional antiepileptic therapy. Myoclonus is another common finding in patients with Alzheimer's disease, occurring in about 10 of autopsy-verified cases, and is a late manifestation. Non-convulsive status epilepticus can occur, and is often overlooked. An EEG is helpful in diagnosis, and should be considered in any demented person whose condition acutely deteriorates. Seizures occur in the other common dementing illnesses also, and are particularly common in cerebrovascular disease (see p. 51). As the population ages, the...

Cortical Circuitry and Alzheimers Disease

In the absence of AD, there does not appear to be a significant neuron loss in the entorhinal cortex in aged humans (Gomez-Isla et al., 1996 Hof et al., 2003 West, 1993), nor is there loss of neurons in the entorhinal cortex in aged monkeys (Gazzaley et al., 1997). However, a lack of quantifiable neuron loss does not necessarily mean that degenerative changes are not occurring in the entorhinal cortex as humans age. In fact, virtually all humans over the age of 55 have some NFTs in layer II of the entorhinal cortex (Bouras et al., 1994), making it difficult to distinguish qualitatively between age-related degenerative events in the entorhi-nal cortex that represent early progressive AD and those that are more stable. Many of the NFTs in this region in normal adults or patients with mild cognitive impairment (MCI) are ''transitional neurons,'' that is, neurons that are still intact and are included in an analysis of total neuron counts, yet have transitional intraneuronal pathology...

Neurological disease in which both dementia and seizures occur

It is well recognized that symptomatic seizures occur in the context of dementia syndromes in older people (Forsgren et al., 1996). Breteler et al. (1995) found that people aged 50-75 with a diagnosis of epilepsy had an overall relative risk of 1.5 of subsequently developing a dementia, which they described as a moderately increased risk over the expected rate. People with Down's syndrome are at particular risk of developing early Alzheimer's disease, and this is frequently associated with seizures (Collacott, 1993 Lott and Lai, 1982). In people with mental retardation (learning disabilities) who do not have Down's syndrome, the incidence and prevalence of dementia is higher than expected, and is often associated with poorly controlled epilepsy (Cooper, 1997). There are also isolated case reports showing an apparent association between seizures, dementia and a pathological lesion. It may well be that there are many such very rare or even one-off disorders (Yerby et al., 1986). One...

Dementia

Dementing disorders are common in the elderly with an overall prevalence in persons 65 years and older of 6 49 and increase exponentially as a function of age in the 65-85-year age range 50 . Alzheimer's disease is the most common cause of dementia in the Western hemisphere, followed by multi-infarct dementia. Compared to non-demented individuals matched for age and sex, patients with Alzheimer's disease have a six-fold heightened risk of unprovoked seizures 51 and people with other types of dementia have an eight-fold increased risk. Seizures may occur as early as 3 months after the diagnosis of Alzheimer's disease or up to 9 years afterwards. The range is even wider in other dementias ( 1 month to 22 years) suggesting a lack of correlation with the duration of the disease. This finding is in contrast with clinical series showing that seizures tend to occur in the late stages of the disease. Partial onset seizures are the prevailing type in Alzheimer's disease and generalized onset...

Niemann Pick disease type C

Middle to late childhood with the insidious onset of ataxia, vertical supranuclear gaze palsy, dementia, dystonia and seizures. The seizures are partial or generalized, or both. The epilepsy is often refractory to medical therapy, but improves if survival is prolonged, presumably reflecting neuronal loss. About 20 of affected children have cataplexy induced by laughing. Dysarthria and dysphagia eventually become disabling, making oral feeding impossible. Adults are more likely to present with dementia or psychiatric symptoms. The diagnosis of NPC is confirmed by bone marrow biopsy which shows lipid-laden histiocytes, the biochemical demonstration of impaired cholesterol esterification and positive filipin staining in cultured fibroblasts. The diagnosis is often delayed substantially because 'routine' screening tests for metabolic disease, such as urine screens and lysosomal enzyme panels, are negative. In about 90 of cases NPC is caused by an identifiable mutation in the NPC1 gene...

Organic acidurias and aminoacidurias

Lactic acidosis is present in blood and urine in Leigh's syndrome. This is usually due to X-linked or autosomal recessive nuclear gene mutations of one of the mitochon-drial respiratory chain complex I or IV enzymes or pyruvate dehydrogenase (X-linked PDHA1 gene). About 30 are due to mutations in mitochondrial DNA, which have been identified in at least 11 mitochondrial genes. The diagnostic features are a subacute encephalopathy, seizures (myoclonic or tonic-clonic), and progressive dementia with cerebellar and brainstem signs. Motor abnormalities include hypotonia, spasticity, ataxia, involuntary movements and bulbar problems. Vomiting, hyperventilation and abnormalities of thermoregulation are common. Optic atrophy, pigmentary retinopathy, deafness and cardiomyopathy are sometimes present. On imaging, basal ganglia lucencies are highly characteristic, and proton magnetic resonance spectro

Surgical Risk Complications And Benefits

Unfortunately, many patients receive misinformation about the dangers of epilepsy surgery, dissuading them from seeking evaluation. In actuality, the death rate from epilepsy surgery is about 1 , with anterior temporal lobectomy having a lower mortality ( 1 ) than hemispherectomy 95 . In a recent report by Engel et al. 77 , 2 out of 556 patients (0.4 ) from seven centres died within a month of surgery, but deaths were unrelated to the surgical procedure. The risk of death related to surgery is likely to be lower than that related to sudden unexpected death in epilepsy (SUDEP), which is estimated to be 0.35 to 4.5 per 1000 patient years, depending on patient profile 96 . One could therefore argue that the risk of surgery is less than the risk of continued, medically refractory epilepsy. Beyond overt mortality, continued seizures have been associated with cognitive decline over time in TLE 97 .

Neuronal ceroidlipofuscinosis

The neuronal ceroid-lipofuscinoses (NCLs) are a group of inherited lysosomal-storage disorders which may present with progressive myoclonic epilepsy and mental and motor deterioration. These are the most common of the hereditary progressive neurodegenerative diseases, occurring generally in about 1 in 25,000 live births, but there is marked geographical variation with a particularly high frequency in Finland. The phenotypes are categorized by age of onset infantile neuronal ceroid-lipofuscinosis (INCL), late-infantile (LINCL), juvenile (JNCL), adult (ANCL), and Northern epilepsy (NE). Myoclonic epilepsy is a feature of all types. Almost all cases are inherited in an autosomal recessive manner although an autosomal dominant form of adult-onset NCL has been described. Carriers show no symptoms. In Santavuori disease (INCL), infants are normal at birth and then develop retinal blindness and seizures by 2 years of age, followed by progressive mental deterioration, and usually death in the...

Conclusions And Implications For Therapeutics

Although many aspects of genotype-phenotype relationships remain poorly understood, recent years have brought considerable progress, encouraging optimism that the pathogenic mechanisms in these disorders can be understood in detail. Examples of such progress are the identification of the role for KCNQ channels in setting thresholds by targeting to the axonal spike initiation zone, the discovery that SCN1A subunits are preferentially expressed on inhibitory interneurons, and the analysis showing how KCNM1 mutations increasing the activity of BK channels can paradoxically increase neuronal excitability (30, 81, 155). However, it remains unclear whether, in GEFS+ families, the variable phe-notype seen within pedigrees may reflect differences in genetic background between individuals, environmental exposures, or both. The spectrum of phenotypes associated with SCN1A mutations, ranging from normal gene carriers or patients with only FS in GEFS+ pedigrees to SMEI patients with severe...

Other Rare Epilepsies Associated with Simple Mendelian Inheritance

Several other less common epilepsies result from simple Mendelian inheritance, such as the progressive myoclonus epilepsies. However, these disorders are exceedingly rare and generally require diagnostic technologies not available in developing world settings. Mitochondrial disorders include myoclonic epilepsy with ragged red fibers (MERRF) and mitochondrial encephalomy-opathy with lactic acidosis and stroke-like episodes (MELAS). The overlapping features of mitochondrial disorders include seizures, dementia, progressive external ophthalmo-plegia, sensory neural hearing loss, cardiac abnormalities, and elevated levels of lactate and pyruvate.

Differential diagnosis

The specific syndromes of myoclonic astatic epilepsy (MAE) and SMEI are also part for the GEFS+ spectrum . MAE presents between 7 months and 6 years, manifests as myoclonic seizures with an associated atonic component, and has a relatively benign natural history with most seizures abating within a few years and with up to 60 of patients being cognitively normal . This is in contrast with SMEI, which presents with prolonged febrile seizures, followed by the development of myoclonic, atypical absence, and sometimes focal-onset seizures associated with variable cognitive decline in the majority of patients

Definitions and context

As Lishman (1998) has pointed out, the term 'dementia' has two potential meanings in medical practice first, it may refer to a group of specific diseases, and second, it is used to describe a clinical syndrome that can have many causes. Although diseases in the former group are characterized by irreversible decline in function, the latter includes conditions in which decline can be arrested, or in some cases reversed. Both ICD-10 (World Health Organization, 1992) and DSM-IV (American Psychiatric Association, 1994) offer detailed diagnostic criteria for the syndrome, with DSM-IV defining additional principles for diagnosing different varieties of dementia. Both of these, in attempting to ensure uniformity of populations that might be used for research purposes, adopt a 'cookbook' style of approach. In this chapter, the term is used in the second of the meanings described by Lishman. 'Dementia' is regarded as an acquired global impairment of intellect, memory and personality, which is...

Refractory Epilepsy A Progressive Intractable but Preventable Condition

Summary The authors propose a hypothesis for the conceptual understanding and prevention of refractory epilepsy based on accumulated laboratory findings and an improved knowledge of the natural history of treated epilepsy. Refractory epilepsy can be recognized as a distinct condition with multifaceted dimensions, including neurobiochemical plastic changes, cognitive decline and psychosocial dysfunction, leading to dependent behavior and a restrictive lifestyle. The biological basis of refractoriness may be multifactorial, and may include the severity of the syndrome and or underlying neuropathology, abnormal reorganization of neuronal circuitry, alteration in neurotransmitter receptors, ion channelopathies, reactive autoimmunity, and impaired antiepileptic drug (AED) penetration to the seizure focus. Recurrent seizures may be the cause of some of these changes. The authors hypothesize that refractory epilepsy may be prevented by interrupting this self-perpetuating progression....

Possible relationships

As Gowers implied, dementia and epilepsy may both be consequences of the same underlying disorder, rather than one being a consequence of the other. Some specific clinical epilepsy syndromes are associated with acquired disorders of intellect and memory, although it is not clear whether seizure activity is responsible for the cognitive changes or whether, like the previous group, there is a shared aetiology. It is however known that individual seizures may result in a cognitive penalty, and that interictal epileptiform EEG discharges can sometimes disrupt cognitive functioning. Some antiepileptic drugs can also play a part. These potential relationships are considered in turn.

The Treatment Of Epilepsy In The Elderly

Epilepsy is a frequent and generally under-recognized problem in the elderly. Annual incidence rates in a recent study were 87 per 100,000 in the 65-69 age group, 147 per 100,000 of people in their 70s, and 159 per 100,000 of people in their 80s, and about 30 of new cases now occur in people over 65 years of age. The prevalence rate of treated epilepsy in those over 70 years is almost double that in children. Currently, about 0.7 of the elderly population are treated for epilepsy, and epilepsy in the elderly is now the third most common neurological condition after dementia and stroke. As the number of elderly people in the population is rising, the numbers of elderly people requiring treatment for epilepsy is also greatly increasing. The medical services must catch up to make adequate provision.

Crosssectional studies

There are different approaches to infer cognitive changes along the time axis. From a scientific as well as methodological point of view, prospective longitudinal studies are generally superior to cross-sectional studies. Clinical observations and results of longitudinal studies suggest that there is, if any, no rapid cognitive decline in patients with refractory TLE. Consequently, longitudinal studies in TLE patients are confronted with the problem of probably small effect sizes. Hence, to provide statistical evidence, large samples of patients have to be observed over long periods of time, probably exceeding decades. In contrast, cross-sectional studies allow the recruitment of large sample sizes. However, the existence of a duration effect only can be inferred from interindividual differences in the duration of illness. Hence, the interpretation of duration effects strictly presupposes that the patients were recruited from the same population. Otherwise a cohort bias might...

Implications For Epilepsy And Conclusions

The previous sections have shown that experimental evidence and computational modeling studies support a role of synaptic and intrinsic homeostasis mechanisms in stabilizing cellular and network activity while allowing network plasticity through mechanisms such as LTP and LTD. But we have also seen that, if not properly constrained, homeostatic regulation can lead to network instability and dysfunction. It is tempting to speculate that failure or malfunction of regulation mechanisms that normally support stable network function in the intact and healthy brain (Debanne et al., 2003) could contribute to network instabilities such as over-excitation or seizure-like activity in diseased or injured brain structures (see also Chapter 26). For example, exposure to a single episode of status epilepticus leads to activity-dependent upregulation of T-type calcium currents in a rat model of epilepsy, which in turn increases the tendency to burst firing in hippocampal pyramidal neurons that is...

Epidemiologyaetiology

Figure 10.1 details the incidence of epilepsy according to age 2 . It is interesting to note that epilepsy has a peak incidence in older age groups. The annual incidence of epilepsy is 134 per 100 000 in those aged 65 years or older 2-7 . In contrast, the incidence of Alzheimer's-type dementia is approximately 123 per 100 000 8 . Epilepsy increases with age, with an exponential increase in incidence for every decade of life after the age of 65 2 . Thus, a seizure in the older adult is more often the rule than the exception.

Epilepsy Risk Factors In The Elderly

There are several risk factors that can increase the potential for seizures in older adults. These include stroke dementia of all types trauma, particularly with haemorrhage (three- fold) alcohol usage infection low-grade neoplasms and depression 2, 9-19 . Of these risk factors, cerebrovascular diseases, neurodegenerative conditions such as Alzheimer's dementia, and low-grade neoplasms are frequently associated with seizures in older adults and should always be considered in the differential diagnosis in patients who are having repeated spells.

Pharmacokinetic Considerations in Pediatric and Geriatric Age Group

The general principles of AED treatment for the elderly are similar to those for other adults, but some differences in the pharma-cokinetics need to be remembered when treating the elderly. Older patients are at risk of having toxic AED levels because of reduced hepatic metabolism and reduced renal clearance of certain AEDs. This population is also at greater susceptibility to cognitive and other neurotoxic side effects due to concurrent illnesses like Alzheimer's disease. Reduced protein binding and hypoal-buminemia can lead to increased free fraction of highly bound drugs. Most of the elderly population will be on other medications for various medical problems, and drug

Electroencephalography

Evaluation of older patients with electroencephalography does not differ from that of younger patients however, there are a number of EEG changes that are commonly seen in the elderly but are not indicative of epilepsy. These changes include slowing of the background alpha rhythm accompanied by a decrease in amplitude. Slow EEG frequencies such as delta and theta can increase with age 34-42 . Slowing of the background is often non-specific when it occurs diffusely, but it may implicate a central nervous system (CNS) structural lesion if it occurs focally. EEG patterns of uncertain significance can occur with age, such as small sharp spikes, or subclinical rhythmic electrical discharges of adulthood (SREDA), the latter occurring exclusively in older adults 40, 41 . Of note, epileptiform transients can be seen in patients with diseases other than epilepsy such as dementia, stroke, neoplasms and prion diseases 43 . Nevertheless, capturing epileptiform transients on an EEG in a patient...

Other Benefits of VNS Therapy

Beneficial clinical effects beyond changes in seizure frequency have been observed. Malow and colleagues84 found that in 16 patients treatment with low stimulus intensities improved daytime sleepiness and promoted alertness. The use of VNS Therapy to reduce morbid obesity,85 86 improve memory,87 improve treatment-resistant depression 8 and to elevate mood59-91 have been reported. Pilot studies investigating the role of VNS Therapy for Alzheimer's disease, obsessive-compulsive disorder, migraine headaches, and anxiety disorders are underway.

Seizures in Later Life

Summary Seizures in Later Life, a videotape, is for older persons with epilepsy, their families, and others interested in the impact of epilepsy on senior citizens. Epilepsy and seizures are as likely to begin occurring in a person's 60's, 70's, or 80's as they are in the first 10 years of life there are about 300,000 older Americans with epilepsy today. People can develop seizures in later life due to head injuries, brain tumors, stroke, or for no apparent reason. The videotape features a neuropsychologist and a clinical pharmacist who help educate people about the nature of epilepsy in later life including causes, treatment, and the appropriate response to seizures. Four older persons share what they have learned about coping with lifestyle changes, safety issues, and overcoming negative feelings about epilepsy. Persons with epilepsy must know that medicines can produce side effects and these should be reported to their doctors. Complex partial seizures are the most common in older...

Treatment And Outcome

The patient underwent a right temporal lobectomy. Pathological examination of the resected tissue was consistent with hippocampal sclerosis. During the year that elapsed after our initial meeting, her father was diagnosed with Alzheimer's disease, her mother developed breast cancer, and a close male friend was diagnosed with testicular cancer.

Brain Mechanisms in Combined RBDExtrapyramidal Disorders

The retrorubral nucleus is located near the substantia nigra, and appears to be implicated in the linked PD-RBD pathology (Lai and Siegel, 1990). The retrorubral nucleus projects to the caudate and putamen (extrapyramidal motor system) experimental lesions to the retrorubral nucleus in cats releases abnormal motor activity during both sleep and wakefulness, ranging from myoclonic twitches to rhythmic leg movements and locomotion (Lai and Siegel, 1997). In addition, the substantia nigra also is closely connected to the REM-phasic generator circuitry and may play a major role in the genesis of PGO waves, a characteristic REM sleep phasic event (Datta et al, 1991). In regard to MSA, pontine involvement has been revealed by both gross neuropathological examination and histo-chemical studies, as cited by Plazzi et al. (1997). Functional magnetic resonance brain imaging studies and postmortem brain analyses are required to definitively elucidate the underlying neuropathology...

De novo absence status of late life

This curious syndrome presents in late adult life. The leading symptom is confusion, although the other features of absence status can occur. Many patients have a history of absence epilepsy in early life, but which has been in long remission. Many cases are misdiagnosed as dementia or cerebrovascular disease, but the abrupt onset should suggest the possibility of absence status, and the diagnosis is easily confirmed by EEG. In most cases, psychotropic drug (particularly benzodiazepine) toxicity or withdrawal seems to be the antecedent cause of the episode. The condition is rapidly alleviated by intravenous lorazepam (4 mg) and tends not to recur. Long-term antiepileptic drug treatment is not usually required.

Silver Spring MD 209078218 Vocabulary Builder

Dementia An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is

Unverrichtlundborg Disease

The myoclonus usually is quite severe and may be precipitated by movement, stress, or sensory stimuli. Repetitive morning myoclonus is also typical, frequently building up and culminating in a major tonic-clonic seizure (7, 8). Seizures may be difficult to control, but progression in terms of ataxia and dementia is mild and late. The clinical course is variable, and there may be considerable intrafamily variation in the severity of the seizures. Some patients are relatively mildly affected and survive to old age. Others have a more fulminant course, with death within a few years of onset this outcome seems

Myoclonus Epilepsy With Ragged Red Fibers

Myoclonus epilepsy with ragged red fibers was first described in cases with a florid clinical myopathy and myoclonus epilepsy (22, 23). It is now clear that the clinical spectrum of MERRF is extremely broad. It should be suspected in a wide variety of situations, even when clinical and pathologic evidence of myopathy are absent (24). Symptoms may begin at any age, and there may be marked intrafamily variation in the age of onset and clinical severity (24, 25). The clinical features include myoclonus, tonic-clonic seizures, dementia, and ataxia, with less common findings of myopathy, neuropathy, deafness, and optic atrophy. Some cases show striking axial lipomas. Occasional patients or families have focal neurologic events, and there is an overlap with the syndrome of mitochondrial encephalomyopathy, lactic acido-sis, and strokelike episodes (MELAS), in which strokelike

Aetiology And Severity Of Intellectual Disability

The aetiology of the intellectual disability may provide several potentially useful management pointers to further understanding of the epilepsy or the behavioural and physical morbidities the individual may experience. An example of useful markers can be seen in some genetically determined conditions associated with intellectual disability. Angelman's syndrome shows a seizure type believed to be of a cryptogenic generalized nature 5 whilst Fragile X syndrome shows features similar to benign epilepsy with centrotemporal spikes 6 . Epilepsy occurs in 5-10 of people with Down syndrome and has a bimodal age distribution for seizure onset. The first peak is during childhood and presents as infantile spasms, myoclonic, atonic and tonic-clonic seizures. Partial seizures are rarely seen 7 . The second peak is in later life, usually as myoclonic or tonic-clonic seizures, and is often associated with Alzheimer's disease. Our ability to recognize relatively well-defined epilepsy phenotypes in...

Mitochondrial Encephalomyopathies

MELAS has among its defining features strokelike episodes, typically before age 40 encephalopathy characterized by dementia, seizures, or both and evidence of a mitochon-drial myopathy with ragged red fibers, lactic acidosis, or both. A variety of point mutations have been described that are causal, though one mutation accounts for about 80 of cases (115). Patients with MELAS frequently have seizures. In one group, seizures were the initial clinical symptom in 28 . They were sometimes associated with a strokelike episode. Seizures were both generalized and partial (116). Partial seizures were most typically motor. Seizures typically respond to conventional antiseizure agents, though valproate may exacerbate the disorder (117).

Inflammatory and immunological diseases of the nervous system

Eration, and sometimes dementia, is another characteristic neurological syndrome. Coeliac disease may also cause cerebral vasculitis. A recent study, however, suggested that epilepsy was no more common in coeliac disease than in the general population. Paraneoplastic disorders can cause seizures. Epilepsia partialis continua (EPC) can occur as a paraneoplastic phenomenon. This is usually in the context of a paraneo-plastic 'cortical encephalomyelitis' due to various tumours (notably small cell lung cancer and breast carcinoma). The MRI scan can show non-specific T2 signal changes. Seizures also occur in limbic encephalitis of paraneoplastic origin often associated with memory loss, psychiatric and behavioural changes.

Longterm irreversible effects

The discussion about irreversibility of seizure-related cognitive effects thus concentrates on cases with ongoing seizure activity and consequently on factors such as seizure duration, number of seizures before treatment (Camfield et al., 1996), number of seizures during lifetime (Dodrill, 1986) or number of years with seizures (Jokeit and Ebner, 1999). This is very reminiscent of Gowers' suggested mental decay ('epileptic dementia') as a consequence of the pathological long-term

Diagnosing The Specific Type Of

Although patients with the PME syndrome superficially may appear to have similar clinical features, knowledge of the specific clinical patterns of the common causes of PME often allows the differential diagnosis to be narrowed. Age at onset of symptoms provides some guidance in making the diagnosis, although MERRF may begin at any age. Certain seizure patterns are helpful very prominent myoclonus suggests Unverricht-Lundborg disease, MERRF, or sialidosis. Partial seizures, particularly of occipital origin, can occur in a variety of the disorders but are often noted in Lafora disease. Characteristic fun-dal changes are almost invariable in sialidosis and are frequent in the NCLs. Dementia is a constant feature of Lafora disease and NCLs, whereas it is characteristically absent or mild in Unverricht-Lundborg disease and sialidosis type I. The presence of deafness, lipomas, optic atrophy, myopathy, or neuropathy are clinical pointers to MERRF. Neuropathy may also occur in sialidosis....

Life And Death Of Neurons In The Aging Cerebral Cortex

Cortical Circuitry and Alzheimer's Disease The transition from age-associated memory impairment (AAMI) to the dramatic loss of cognitive abilities accompanying Alzheimer's disease (AD) requires progressive development of neocortical pathology that results in neuron death. The selective vulnerability of this neuron death is reflected in the characteristics of cortical pyramidal neurons that are prone to form neurofibrillary tangles. Loss of the neurons that form long corticocortical projections in the association neocortex emerges as the pathological outcome most directly related to the dementia observed in AD. AAMI likely involves alterations of neuronal spines and synapses without neuron death. Interestingly, the same circuits that are vulnerable to degeneration in AD are vulnerable to synaptic alterations short of neuron death. These synaptic alterations likely impact cognitive function in normal aging in a manner consistent with the more modest cognitive decline typically seen...

Temporal Gate hypothesis

In other neurological conditions associated with deterioration, the phenomenon of oxidative stress (the production of oxygen radicals beyond a threshold for proper antioxidant neutralization) has been implicated. These include Alzheimer's disease (Sims, 1996), Parkinson's disease (Jenner and Olanow, 1994), amyotrophic lateral sclerosis (Gorman et al., 1994), Pick's disease (Castellani et al., 1995J and schizophrenia (Ramchand et al., 1996). Various intracellular messenger systems involving glutamate are implicated in oxidative radical production. These systems are involved in neuronal growth, differentiation and apoptosis (Michaelis, 1998). Glutamate is also known to play an important role in epilepsy. The author has observed substantial improvement in cognitive functioning in two patients (one male 'subcortical', one female 'temporal') after using lamotrigine, a glutamate release inhibitor (Meldrum, 1994). There are various other reports, generally of an anecdotal nature, of the...

Is Aed Treatment Indicated After The First Unprovoked Seizure The First Trial Group

Neuropsychological studies showing cognitive decline over time in patients with uncontrolled seizures 13-15 , and serial imaging studies demonstrating progressive loss of cerebral volume in certain subgroups of patients with epilepsy 16, 17 would appear to support the view that epilepsy is a progressive condition, at least in some patients. However, studies in drug-naive patients in developing countries, where AED treatment has not been readily available, paints a different picture. In their study of treatment outcomes in patients in Kenya, Feksi and colleagues found that even patients who had suffered over 100 generalized seizures had remission rates comparable with patients who were treated after a few seizures 18 . Moreover, epidemiological studies examining the incidence and prevalence of epilepsy have consistently found prevalence rates much lower than would be expected from cumulative incidence rates. This suggests that a significant proportion of epilepsy does enter remission...

Head Trauma Stroke and Degenerative Brain Disorders

Tries, mainly due to the relatively younger age of the population. However, an increase in the average life span in some developing countries, coupled with Westernized diets and increased tobacco use, have resulted in an increase in cerebrovascular accidents and dementia in these areas. Roughly 15 of patients with Alzheimer's disease (AD) experience unprovoked seizures in the course of their illness. In industrialized countries, AD accounts for almost 15 of all newly diagnosed cases of epilepsy in the elderly (after the age of 65). There is an urgent need to have reliable data on the occurrence of epilepsy after head injury, stroke, and degenerative brain disorders among populations living in developing countries. Such information would assist with public health interventions to prevent epilepsy and health services planning.

Distinguishing Pme From Other Epilepsies And Myoclonic Syndromes

This is particularly so in those forms of PME associated with relentless dementia, such as Lafora disease and NCL. Generalized epileptiform abnormalities are seen during the resting record, usually in the form of fast spike-and-wave, multiple spike-and-wave, or multiple spike discharges. Photosensitivity is common and may be marked. Focal, particularly posterior, epileptiform abnormalities are common in Lafora disease but also may occur in other forms (11). Somatosensory evoked potentials (SEPs) frequently show giant responses (66). PMEs should be distinguished from degenerative disorders in which seizures and or myoclonus can occur but do not form part of the clinical core or usual initial presentation of the disorder. The causes of such progressive encephalopathies with seizures are numerous and include GM2 gangliosidosis, nonketotic hyperglycinemia, Niemann-Pick type C, juvenile Huntington's disease, Alzheimer's disease, and so forth. The distinction between this...

Dentatorubropallidoluysian atrophy DRPLA

Dentato-rubro-pallido-luysian atrophy is inherited in an autosomal dominant fashion. It occurs with markedly varying frequency around the world, being particularly common in Japan (a frequency of 0.2-0.7 per 100,000 persons) and in northern Europe. It is a triplet repeat disorder involving the DRPLA gene, which is of uncertain function. The normal repeat number is between 6 and 35 and the condition is present with full penetrance when the repeat number is greater than 48. DRPLA is a slowly progressive disorder in which ataxia, choreoathetosis, dementia and behavioural changes, myoclonus, and epilepsy occur. In common with other triplet repeat disorders, significant 'anticipation' is exhibited, and there is marked phenotypic variation even within a family. Fifty per cent of cases present tonic-clonic seizures and myoclonus, some present with ataxia-athetosis-chorea, and others with dementia in a 'pseudo-Huntington' form. The condition can present at any time from infancy to late adult...

NEuRoprotection In StatuS EpilepticuS

As discussed, prolonged seizures may result in neuronal death, which in turn may lead to cognitive decline and epilepsy. As the mechanisms underlying neuronal death in SE become better understood, research has focused on methods of preventing the pathophysiological changes that result in neuronal damage. Thus, most potential neuroprotective agents have targeted excitotoxic pathways, including blockade of glutaminergic NMDA receptors and calcium channels or, alternatively, apoptotic pathways. The data on these agents come from various animal models of SE and are summarized in Table 14.3. No drug is currently approved for use in humans as a neuroprotective agent in SE.

Epilepsy secondary to multiorgan hereditary disorders

One of these, Rett's syndrome, is characterized by autistic dementia, gait apraxia, stereotyped hand movements, and bizarre attacks involving hyperventilation, limb and truncal jerking and profuse sweating. Likewise, seizures are a frequent clinical feature in patients with Huntington's disease starting in childhood or adolescence or with Wilson's disease with juvenile onset. Other neurodegenerative disorders cause progressive myoclonus epilepsies, a group of epilepsies of various aetiologies, characterized by myoclonic seizures, tonic-clonic seizures and progressive neurological deterioration with ataxia and dementia 32 , Progressive myoclonus epilepsies account for approximately 1 of cases in referral centres and are mostly due to inherited metabolic abnormalities 33 . Series from different countries reveal marked geographical and ethnic variability in the occurrence of the specific subgroups of progressive myoclonus epilepsies. There are no data on the incidence and prevalence of...

The Psychoses of Epilepsy a Neurological Disease

A close association between psychoses and epilepsy has been known since 1854 confirmed by the fact that the early mental hospitals in Europe had special wards dedicated to epilepsy. The early proponents of psychosis of epilepsy (POE) included Kraepelin (1918) who believed that the dementia resulting from epilepsy was different from dementia praecox or schizophrenia. This was further affirmed by Vorkastner (1918) and Krapf (1928) who clarified that schizophrenialike symptoms might follow epilepsy and that these need to be differentiated from true schizophrenia. Contrary to this, Glaus (1931) and Gruhle (1963) opined that the schizophrenic symptoms of epilepsy could be true independent schizophrenia. In 1860, with the description of alternating psychosis, this relationship underwent some radical thinking, and sadly petered out until the 1950s when the term schizophrenia-like psychosis of epilepsy (SLPE) was coined by Slater et al. (1963). Kraepelin, B. (1918). Dementia Praecox. (R. M....

Movement control structure

The neodissociative model of hypnotic behaviours also provides the basis for an account of more pathological phenomena such as dissociative amnesia, fugue and multiple personality disorder (Kihlstrom, 1994 Spiegel and Cardena, 1991). According to such a view, the formation of amnesic barriers within the executive ego is a common defensive response in the face of trauma. These barriers serve an adaptive function in that they protect the individual from experiencing potentially overwhelming negative affect associated with the traumatic event. However, pathological dissociative amnesia can arise if the barrier within the executive ego endures to the point where the memory loss itself becomes distressing or debilitating. In the case of dissociative fugue, the amnesic barrier conceals large tracts of autobiographical memory as well as the traumatic events themselves. Without access to this autobiographical information, the fugue sufferer not only reports amnesia but also

Why has demonstration of the epileptic nature of this nocturnal bizarre dyskinesia proved to be such a hard task

His spells of complex, bizarre motor phenomenology arising in early stages of NREM were the hallmark for a tentative diagnosis of the so-called NPD but with otherwise non-contributive video scalp-derived EEG or neuroimaging. When these episodes became pharmacologically uncontrolled and he showed evidence of cognitive decline, it was decided to proceed with an invasive video-EEG polygraphy. The problem then was what areas of his brain should be monitored in the hope of discovering the cortical source of the NPD. The perusal of the literature had shown that there are quite discordant opinions and very few data regarding the epileptogenic locus for such seizures. A majority of the authors had favored the frontal lobe as the generator of NPD attacks, but without specifying in which region of the lobe it might be located.31,34,37,41,45,46,68, 75,99,100,101 Some did focus on specific cortices, mentioning the SMA as the possible site for the epileptogenesis of NPDs but without confirmations...

Mitochondrial cytopathy myoclonic epilepsy with ragged red fibres [MERRF

The mitochondrial cytopathy that typically causes progressive myoclonic epilepsy is the syndrome of myoclonus epilepsy with ragged red fibres (MERRF). This is a multisystem disorder with a very variable phenotype, in which myoclonic seizures are often the first symptom, followed by generalized epilepsy, myopathy, ataxia and dementia. Other features are short stature, deafness, optic atrophy, retinopathy, ophthalmoparesis and cardiomyopathy with

Phenobarbital

Phenobarbital is the 'grandfather of anti-epileptic drugs'. It has been available for over 100 years. It is effective for most seizure types and the fact that an intravenous (i.v.) formulation is available means it is often used for treatment of status epilepticus. In the modern era, it is rarely used as first-line therapy, as studies have demonstrated that it causes more dose-related side-effects, particularly sedation, than other options 14 . Also, once started it is very difficult to withdraw without causing seizure exacerbation. Abrupt withdrawal is not recommended, and even slow withdrawal can lead to problems. The initial dose is 30-50 mg, which is best administered at bedtime. Titration to optimal dose can be achieved over several weeks. Optimal effect is usually achieved at serum concentrations of 15-45 mg l. Other dose-related side-effects include irritability, difficulty concentrating, memory loss, sedation, dysarthria and ataxia. Other reported adverse reactions include...

Neuropsychology

And quantifies cognitive function in terms of intelligence, language, memory, perception, and executive function. In patients with epilepsy, neuropsychologists can aid diagnosis, evaluate medication effects, monitor cognitive decline, and make key observations in pre- and postoperative surgical evaluations. Neuropsychologists can also help the assessment of nonepileptic attack disorders, eliciting precipitating factors and ultimately facilitating their management and continuing care.

Elderly

The age-specific incidence of epilepsy is U shaped, with neonates and the elderly showing the highest rates. Most (75 ) elderly-onset epilepsy is explained as being due to cerebrovascular disease17 other causes include tumors, dementia, and head trauma. The diagnosis is again entirely clinical, relying upon history and eye-witnessed accounts. However, such detailed descriptions may be less achievable in frail elderly people living alone. Furthermore, comorbidities such as ischemic heart disease and cognitive impairment and polypharmacy can make diagnosing epilepsy in an elderly person a considerable challenge. An epilepsy diagnosis carries immense medical and social implications for an elderly person, including risk of injury, confusion, depression, fear of isolation, and lack of independence. Furthermore, an underlying ischemic-related arrhythmogenic cardiac syncope (easily attributed to epilepsy) may easily prove fatal, yet the misdiagnosis of epilepsy may remain unrecognized even...

Down syndrome

The Down phenotype occurs in about 1 in every 650 live births. It is usually caused by trisomy of chromosome 21, and triplication of 21q22.3 results in the typical phenotype. In 95 of cases the cause is a non-dysjunction, and in about 4 an unbalanced translocation. About 1 of cases are due to mosaicism. The risk of trisomy increases with maternal age. Epilepsy is present in up to 12 of cases and EEG abnormalities in more than 20 . Epilepsy typically develops either in the first year of life, due to perinatal or congenital complications, or in the third decade, possibly due to the development of Alzheimer-like neuropathological changes. The epilepsy is very variable and can take multiple forms, reflecting the complex pathogenesis. West syndrome is common, and Down syndrome can also cause febrile seizures and Lennox-Gastaut syndrome. Usually the epilepsy is rather non-specific, although frequent, small, brief, partial seizures seem particularly common in adults. Startle-induced...

Hemimegalencephaly

This term describes a gross structural abnormality which can be the end result of various cerebral processes and insults. One cerebral hemisphere is enlarged and is structurally abnormal with thickened cortex, reduced sulcation, and poor or absent laminar organization. Giant neurones are found throughout the brain and in 50 of cases balloon cells also. The condition can occur in isolation, associated with other cortical dysplasias or as part of other syndromes (notably tuberous sclerosis or other rarer neurocutaneous syndromes such as epidemal naevus syndrome, Klippel-Trenaunay-Weber syndrome, neurofibromatosis type 1 or hypomelanosis of Ito). The restriction of the abnormality to one hemisphere may be due to somatic mosaicism, and it has been suggested that the condition is due to defects in the process of programmed cell death (apoptosis) in early fetal life. Hemimegalencephaly always results in severe epilepsy presenting in early life, accompanied by learning disability, hemiplegia...

Febrile Seizures

Risk factors for epilepsy vary depending on the age of the individual. Children, and particularly infants, appear to have a lower seizure threshold than adults (including the FS phenomena exclusively seen in children), but may be more resistant than adults to the development of recurrent, unprovoked seizures. For example, children are more likely to have a seizure after head injury, but are less likely than adults to develop epilepsy after head injury. Epilepsy in adults occurs most often in the elderly and reflects the higher incidence of CNS injury in this population due to stroke, dementia, and other neurodegenerative processes. The location of CNS injuries also impacts the risk of recurrent seizures, with the temporal and frontal regions appearing to be most epileptogenic and the occipital and subcortical regions less so. Recognizing that in at least 30 of people with epilepsy, no underlying cause can be identified even with state-of-the-art imaging and technology, several clear...

Figure

Several antagonists of the NMDA receptor channel complex have been developed, some of which block the NMDA receptor (competitive NMDA antagonists) and others that block the associated cation channel (noncompetitive antagonists). Drugs such as acid (CPP), CGS-19755, and 2-amino-5-phosphonovaleric acid (AP5) are competitive blockers at the NMDA receptor (23). The channel binding site is sometimes referred to as the phencycli-dine receptor. Two potent preclinical noncompetitive channel-blocking drugs are MK-801 and phencyclidine (24, 25). The clinically approved anesthetic ketamine (26) and the antitussive dextromethorphan (27), as well as the anticonvulsant felbamate, are also NMDA channel blockers. Memantine, an un-competitive NMDA channel blocker, has recently been approved by the Food and Drug Administration for use in Alzheimer disease. It differs from noncompetitive NMDA blockers in that it binds more avidly to open channels, rather than indiscriminately blocking all channel...

Cerebral damage

Another contentious issue is the extent to which seizures can result in cerebral damage or produce progressive motor, sensorial or cognitive impairment. The consensus view currently is that cerebral damage from short self-limiting seizures is extremely unusual, and prospective studies have generally failed to demonstrate any significant brain damage. The risk after prolonged seizures (e.g. status epilepti-cus) is quite a different matter, and severe status can result in significant cerebral damage and consequent cognitive decline. This is partly owing to the underlying cause, but there seems little doubt that the epileptic activity itself results in neuronal death and gliosis. The risk is probably greatest in children, and the longer the convulsive phase, the higher the risk of brain damage. In some of the childhood epilepsy syndromes there is evidence that ongoing epilepsy results in cerebral damage, for instance in the Sturge-Weber syndrome, in West syndrome due to tuberous...

Inspection

Muscular pseudohypertrophy is a feature of disseminated cysticercosis. Patients with disseminated cysticercosis present with uncontrolled seizures, progressive dementia, behavior disorder, muscular pseudohypertrophy, and a relative paucity of localizing neurological signs or signs of raised intracranial pressure.52 Most of the reports of disseminated cysticercosis with muscular pseudohypertrophy have been reported from India.53-58

Conclusion

A., DelCastillo-Castaneda, C., Jacobs, D. M., Marder, K., Bell, K., Albert, M., Brandt, J., and Stern, Y. (2006). Incidence and predictors of seizures in patients with Alzheimer's disease. Epilepsia 47, 867 872. Banh, H. L., Burton, M. E., and Sperling, M. R. (2002). Interpatient and intrapatient variability in

REM Sleep Disorders

Behavioral management and treatment of any comorbid medical conditions are the appropriate treatment strategies. The onset of a REM behavior disorder may rarely be the first clinical sign of a brainstem lesion, and neuroimaging may be appropriate. In adults REM behavior disorders and acting out of dreams, sometimes violent, may predate by many years other symptoms of serious neurological disorders such as Parkinsons disease, dementia with Lewy bodies, or multiple system atrophy.51

Historical aspects

'dementia' acquired a medical connotation in the second half of the eighteenth century. He quotes the French encyclopaedia of 1765, which lists as causes 1. damage to the brain caused by excessive usage, congenital causes or old age, 2. failure of the spirit, 3. small volume of the brain, 4. violent blows to the head causing brain damage, 5. incurable diseases such as epilepsy, or exposure to venoms . . . Dementia is difficult to cure as it is related to damage of brain fibres and nervous fluids it becomes incurable in cases of congenital defect or old age . . . otherwise treatment must follow the cause. (Diderot and d'Alambert 1765) The mind is slowly weakened by the storms of fury through which it passes, and they sink finally into the apathy of dementia - a state of mere oblivion, in which they cease to hope or care more. (Maudsley, 1874) observations regarding the effects of concomitant brain disease, recurrent head injury due to seizures, and undesirable effects of antiepileptic...

Treatment effects

A dementia-like picture sometimes seen with phenytoin has been long recognized (Rosen, 1968) this may be related to the serum level of the drug (Matthews and Harley, 1975) or to phenytoin-induced folate deficiency (Neubauer, 1970). In the latter case, folate supplementation can result in improved cognitive performance (Froscher et al., 1995). Phenytoin has also been implicated in thiamine deficiency, resulting in discrete performance deficits in visuo-spatial analysis, visuo-motor speed and verbal abstracting ability. These improve with thiamine supplementation (Botez et al., 1993). Phenytoin has also been reported as causing a specific memory impairment (Butlin et al., 1984 Gillham et al., 1990), most

Figure 1310

Deficits that commence between 14 months and 14 years of age. Fluctuating deficits follow seizures, lengthen, and then become permanent in more than half of affected children. Partial and generalized seizures often evolve over time, and progressive deterioration of neurologic status with hemiparesis, focal neurologic deficits, and dementia characterize the most severe cases. Histopathologically, the affected brain shows diffuse lymphocytic infiltration and perivascular cuffing, consistent with chronic viral encephalitis (32). MRI features have been correlated with quantitative histopathology, showing higher numbers of

Longterm outcome

In the 1960s and early 1970s, the mean survival of ULD patients was 14 years after the onset of symptoms . At present, it is clear that clinical evolution of ULD is greatly influenced by the treatment received Patients today may have a relatively normal life span In appropriately managed patients, dementia can be averted, and myoc-lonus and seizures can be minimized Avoidance of phenytoin is key to this goal Phenytoin is exquisitely toxic to ULD patients and is likely a major contributor to the severity of cases described in the past Still, clinical severity may vary even within families with the same genetic mutation

Samuel F Berkovic

He syndrome of progressive myoc-I lonus epilepsy (PME) consists of myoclonic seizures, tonic-clonic seizures, and progressive neurologic dysfunction, particularly ataxia and dementia. Onset may be at any age but is usually in late childhood or adolescence. Myoclonus in PME is typically fragmentary and multifocal and often is precipitated by posture, action, or external stimuli such as light, sound, or touch. It is particularly apparent in facial and distal limb musculature. Bilateral massive myoclonic jerks, which tend to involve proximal limb muscles, may also occur (1, 2).

Sialidoses

In sialidosis type I ( cherry-red spot-myoclonus syndrome ), there is onset in adolescence with myoclonus, gradual visual failure, tonic-clonic seizures, ataxia, and a characteristic cherry-red spot in the fundus. The myoc-lonus is usually very severe. Lens opacities and a mild peripheral neuropathy with burning feet may occur. Dementia is absent (37, 59).

Rasmussen Syndrome

Have occasional, nondisabling seizures (102, 104, 105). Early hemispherectomy, although increasing the hemi-paresis, reduces the overall burden of the illness because of a marked decrease in both frequency and severity of seizures. Because hemiplegia is inevitable with or without surgery, early surgery may allow the child to return to a more normal life by preventing the cognitive decline that is the result of constant seizures.

Moving Into Humans

Patients undergoing epilepsy resection surgery provide basic scientists a very precious resource for investigation of the cellular processes occurring with seizure disorders. Of particular interest to researchers and clinicians alike, is the notion that distinct and redundant electrical and or chemical patterns may be accessed to deduce ictal onset or method of neuronal recruitment and seizure propagation. Development of rational and effective therapies can be expedited by defining the fundamental mechanisms of seizure and epileptogenesis in humans. As pointed out by Engel (1998), experimentation in surgical patients can prove invaluable in the validation of current animal models, while also providing phenomena specific to the human epileptic brain for the development of new animal models. Building on our experience in non-human primates, we continue to work toward adaptation of the glutamate-sensitive MEA repeatedly utilized in investigations of the mammalian CNS for human...

Sialidosis

Sialidosis is less common than the other causes of progressive myoclonic epilepsy. There are at least two variants. All cases are inherited in an autosomal recessive manner. Type I sialidosis (cherry-red spot myoclonus syndrome) is due to N-acetyl neuraminidase deficiency, which results in defective cleavage, and thus accumulation, of oligosaccha-rides, typically with inclusion bodies with vacuolation. It has a juvenile or adult onset and is characterized by action myoclonus and an intention tremor, gradual visual failure, and later tonic-clonic seizures. There is little in the way of mental deterioration. A gene has been mapped to chromosome 6p21.3. In type II sialidosis there are defects in P-galactosidosis activity in addition to those in N-acetyl neuraminidase. Timing of the onset is variable from infancy to the second decade, and clinical features include severe myoclonus, tonic-clonic seizures, dysmorphic features, coarse facies, corneal clouding, skeletal dysostosis, cardiac...

Potassium Channels

Some anticonvulsant compounds may also play a role as potassium channel openers. Carbamazepine has been found to enhance potassium conductances in neurons (45). Other potential anticonvulsant compounds are being evaluated that may also potentiate potassium channel activation. Investigation of anticonvulsant drugs regulating potassium channels is a major frontier in the development of new anticonvulsant compounds. The small conductance Ca2+-activated K+ (SK) channels inhibit epi-leptiform bursting in hippocampal CA3 neurons. Compounds activating or inhibiting voltage-gated potassium channels formed by KCNQ2, KCNQ3, and KCNQ5 assembly (M-channels) are undergoing clinical trials for epilepsy, stroke, and Alzheimer's disease (46). Mutation in KCNQ2 3 causing mild reduction of M-channel activity is

Antipsychotic drugs

It is perhaps no coincidence that the drug which appears to be the most effective antipsychotic, namely clozapine, is also associated with a high frequency of seizures. The relationship of convulsive seizures to the relief of psychopathology is an integral part of psychiatric therapy, through ECT. It is often forgotten that the latter was introduced for the treatment of dementia praecox, and has clinically and theoretically important antipsychotic effects.

Lafora Disease

Onset of Lafora disease is between the ages of 10 and 18 years, with a mean age of onset of 14 years. Clinical features are myoclonus, tonic-clonic seizures, and relentless cognitive decline. Focal seizures, particularly that arise from the occipital regions, occur in approximately half the patients. Recognition of Lafora disease in its fully developed form is not difficult. At the onset, however, the disorder may resemble a typical benign adolescent generalized epilepsy with no evidence of cognitive decline. It also may present as a dementing illness with relatively infrequent seizures, or it may mimic a nonspecific secondary generalized epilepsy because myoclonus is not obvious (37, 38). The prognosis of Lafora disease is dismal, with death occurring 2 to 10 years after onset and the mean age of death being 20 years. The age of onset, eventual inexorable dementia, and frequent occurrence of focal occipital seizures are clinical clues to the diagnosis (38). Lafora bodies can be...

Lafora body disease

There is also a rapidly progressive and severe dementia. Ataxia and dysarthria also occur. The condition is progressive, although often in a stepwise form, and death occurs within 2-10 years of the onset of the disease, by which time the myoclonus is severe and disabling. The EEG may show spike-wave initially at 3 Hz, with faster frequencies developing over time, and focal occipital spike discharges, although these are not a reliable diagnostic finding. The myoclonus is not associated with EEG change and this helps differentiate early cases from myoclonus in idiopathic generalized epilepsy. Later EEG signs include slowing of background activity and the loss of normal sleep patterns. Up to 80 of patients have a mutation in the EPM2A gene, which codes for laforin, a tyrosine phosphatase protein. A second gene, EPM2B, has been identified, which codes for malin, a ubiquitin ligase protein. A third locus has recently been described in one family. The diagnosis can be confirmed by...

Lewy Body Disease

A subsequent report involved the case of a 73-year-old man with a 2-year history of parkinsonism and a 15-year history of RBD (Negro and Faber, 1996). The clinical history satisfied operational diagnostic criteria for dementia of the Lewy body type (i.e., mix of parkinsonian symptoms, coarse dementia, fluctuating cognitive performance, and intermittent psychotic symptoms). Probable diffuse Lewy body dementia was reported in a 72-year-old man with a 17-year history of RBD and a 2 year history of dementia (Turner et al, 1997). The patient had a typically placid disposition during the daytime, but would attack his wife and attempt to choke her during sleep. Cognitive dysfunction began insidiously, but eventually became the dominant clinical concern. He subsequently developed visual hallucinations and illusions in the late afternoon and evening and often thought his wife was an identical imposter. There was marked fluctuation of cognitive capacities, ranging from relatively lucid...

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