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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and biochemical characteristics of metachromatic leukodystrophy (MLD), true adult forms and late juvenile forms which are still living at adulthood, are reviewed as they both are observed in adult Neurology and Psychiatry departments. Mental deterioration is often the first symptom, evolving progressively; and dementia finally occurs. The latency before the appearance of neurological objective symptoms may be long and extend for several years. In many cases, the behavioral abnormalities are the first symptoms. Some of these forms have been diagnosed as schizophrenia. Very seldom, neurological symptoms, especially ataxia, occur without cognitive or psychiatric disturbances. Most of these cases have pyramidal and cerebellar symptoms, at diverse degrees. Seizures can also occur which is some cases can be early symptoms associated to mental deterioration. The association of central and peripheral neurological symptoms is very characteristic of MLD. The peripheral neuropathy is not generally clinically evidenced, but is rarely missing electrophysiologically. Arylsulfatase A determination should be performed for diagnosis as a first step, and confirmed by the accumulation of sulfatide, either by quantitative determinations in urine or by the sulfatide loading test. It is as yet not clear why certain forms have a rather rapid evolution in 5 years, and others have a very protracted course during decades.
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PMID:Adult forms of metachromatic leukodystrophy: clinical and biochemical approach. 168 76

Subacute sclerosing panencephalitis (SSPE) has become rare since the widespread use of the measles vaccine. In this patient with a 5 month history of seizures and progressive dementia due to SSPE, the atypical electroencephalographic pattern was characterized by generalized spike-and-wave discharges, maximal on the right, associated with clinical seizures. After diazepam (intravenously), more typical periodic complexes appeared.
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PMID:Atypical electroencephalographic pattern in a patient with subacute sclerosing panencephalitis. 170 53

Severe persistent neuropsychological disorders sometimes develop in the course of a focal epilepsy of unknown origin in previously normal children. Very frequent bilateral focal or generalized discharges are often noted on the sleep EEG records of these patients with no evidence of clinical seizures. The relation between these paroxysms and the observed deterioration remains unclear. We report a child with a partial complex epilepsy and severe disturbances of language, cognition, and behavior acquired in the early years of development who was followed for 15 years. A correlation between the evolution of the striking EEG abnormalities during sleep and the neuropsychological disorders could be established retrospectively. The observed sequence of onset and recovery of the aphasia, the dementia, and the "psychotic" behavior makes a direct causal relation between the deficits quite unlikely. Rather it suggests an association of independent symptoms with a specific language disorder becoming manifest in the course of the evolution. This child shows many of the main characteristics of the syndromes of "acquired aphasia with convulsive disorder" (Landau-Kleffner syndrome) and "epilepsy with continuous spike waves during sleep." Both syndromes describe probably different facets of a similar underlying, still unexplained cerebral dysfunction.
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PMID:Acquired aphasia, dementia, and behavior disorder with epilepsy and continuous spike and waves during sleep in a child. 171 72

The present report documents a family with three cases in two successive generations of pigmentary orthochromatic leukodystrophy (POLD). The clinical features of these cases and histochemical and ultrastructural investigations of two of the brains from successive generations are discussed. A review of the familial cases of POLD reported in the literature is also presented. Transmission of these cases was by a dominant inheritance. Onset of the clinical symptoms occurred at 42 to 54 years of age; duration of the disease was from 2-11 years, and death occurred at 45 to 57 years of age. Clinical manifestations of all three cases were severe headaches; bilateral pyramidal, pseudobulbar, cerebellar, and frontal release signs; gait disturbances; euphoria, or apathy; epileptic seizures; and dementia. The neuropathological pattern consists of slight cerebral atrophy, brownish discoloration of the cerebral white matter with demyelination and severe gliosis, sparing the sub-cortical U fibers; presence in the macrophages of lipid pigment granules that are sudanophilic, non metachromatic, and PAS and iron positive. The electron microscopic pattern of the lipid pigment in the macrophages is that of ceroid: electron-dense, membrane-bound intracytoplasmic lysosomes with curvilinear and/or fingerprint profiles.
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PMID:The dominant form of the pigmentary orthochromatic leukodystrophy. 172 27

A 32-year-old woman presented with increasing motor difficulties and memory disturbances. Neurological examination only showed mild cerebellar and extrapyramidal symptoms, whereas neuropsychological evaluation disclosed severe cognitive changes consistent with dementia. Her motor and mental status progressively deteriorated until death, which occurred 5 years after the first admission. One year before death, while she was almost bedridden, symptoms of myoclonic epilepsy first appeared, with frequent generalized seizures and generalized myoclonus, occurring especially upon sensory stimulation or passive joint movements. Pathological examination showed neuronal inclusions typical of Kufs' disease. This case, with primary progressive dementia and late-onset myoclonic epilepsy, differs from previously reported cases. Three special electrophysiological features were abnormal, "giant", evoked potentials; unusually marked photosensitivity; and seizure induction by any sensory stimulation.
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PMID:Kufs' disease presenting as progressive dementia with late-onset generalized seizures: a clinicopathological and electrophysiological study. 173 61

Advances in imaging hardware for positron emission tomography and single-photon emission CT, coupled with a wide variety of radiopharmaceutical agents, have allowed these techniques to be used in the evaluation of neoplasm, stroke, epilepsy, and dementia. Cerebral perfusion agents continue to be the mainstay of single-photon emission CT imaging but, in addition to the evaluation of ischemia, it has seen an increasing role in the study of dementia, neuropsychiatric disorders, and seizures. Positron emission tomography scanning has had similar applications but it is playing a greater part in the evaluation of neoplasms, including primary gliomas and pituitary adenomas. Stable-xenon CT has shown value in the study of ischemia associated with meningitis, sickle cell disease, chronic subdural hematomas, and cerebral arteriovenous malformations. MR diffusion imaging shows promise in the evaluation of white matter pathology and some tumors.
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PMID:Physiologic imaging of the brain. 173 6

We reviewed 18 EEG studies in four members of a family with the Lafora form of progressive myoclonic epilepsy. Each patient was the product of a consanguinous marriage and presented as a teenager with progressive seizures, myoclonus, dementia, and ataxia, and had biopsy proven disease. The EEG early in Lafora disease has spike-wave activity resembling that seen in a primary generalized epilepsy; the background slowing is more typical of a secondary generalized epilepsy. With disease progression, there is increased epileptiform activity, and a striking change in the spike-wave complexes, with a marked increase in frequency up to 6-12 Hz, and many more short duration polyspike components. Unlike some other forms of secondarily generalized epilepsy, the EEG in Lafora disease is distinguished by an increased frequency of the spike-wave complexes with disease progression.
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PMID:Longitudinal EEG studies in a kindred with Lafora disease. 174 63

Thirty-nine cases of primary intracranial arachnoid cyst in the elderly, including one case of our own, were analyzed. Clinical characteristics in these patients were as follows. (1) The number of patients decreased with age, but there were no differences according to sex. (2) Clinical manifestations were similar to those of chronic subdural haematoma or normal pressure hydrocephalus including dementia, urinary incontinence, and hemiparesis. General symptoms such as headache and seizures were also present. (3) Surgery was performed in most patients with generally good outcome regardless of operative procedures (capsular resection vs. shunt). (4) In some cases of advanced age, disease manifestation may have been due to slight head injuries.
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PMID:Primary intracranial arachnoid cyst in the elderly: a survey on 39 cases. 179 42

Twenty seven cases (13 male, 14 female) in the age group of 3-12 years with cerebral cysticercosis were studied and followed up to 3.9 years (mean 1.85 +/- 0.91 years). Seizures (partial 76% and generalized 23.8%) was the feature in 21 patients (77.7%) and raised intracranial tension in 15 (55.5%). Five patients (18.2%) had meningoencephalitis, while 4 (14.8%) had obstructive hydrocephalus due to intraventricular cysts in the 4th ventricle. Twenty two patients received cysticidal drugs (praziquantel or albendazole), while 8 had surgical intervention (CSF diversion, cyst removal, subtemporal decompression or extirpation of the cortical cyst). Mortality was 18.2%. Survivors had epilepsy in 18 patients (81.8%), dementia in 2 (9%), mental subnormality in 6 (27.2%) and hyperkinesia in 12 (54.5%).
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PMID:Neurocysticercosis in children. 180 53

Twenty-five patients with cerebral cysticercosis admitted to the Bangkok Hospital for Tropical Diseases from March 1987 to November 1989 were studied. The patients had a mean age of 41 +/- 5 years with a mean body weight of 57 +/- 4 kgs. Male to female ratio was 1.5:1. Eight patients (32%) gave a history of having taeniasis with a mean duration of 3.6 years before having symptoms of cerebral cysticercosis. Six patients (24%) also had subcutaneous cysticercosis with a duration of 20 +/- 8 months. The important clinical symptoms were headache, focal seizure, epilepsy and dementia. Fourteen patients (56%) had headache, 12 patients (48%) had focal seizure and four patients (16%) had a mild degree of dementia. Baseline study included routine blood examination, biochemical tests, cerebrospinal fluid for routine examinations and immunological study. Biopsy of subcutaneous cysts, plain films of soft tissue and computerized tomography of brain. Praziquantel was given orally at a dosage of 45 mg/kg/day in 3 divided doses at 4-5 hour interval for 15 days. Patients who were taking anti-epileptic drugs before were permitted to continue their medications. The evaluation of results of treatment was done a year post treatment, ten patients (40%) were asymptomatic, 12 patients (48%) had much clinical improvement, their epileptic attack was controlled by 1-2 tablets of phenobarbital (1/2 g) at bedtime. Two patients (8%) had mild headache. One patient (4%) was not improved. Those patients with dementia were not improved.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The treatment of neurocysticercosis with praziquantel. 182 5


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