Cases reported "Seizures"

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1/47. Transient paralytic attacks of obscure nature: the question of non-convulsive seizure paralysis.

    Eleven patients with transient paralytic attacks of obscure nature are described. paralysis could involve face or leg alone, face and hand, or face, arm and leg. The duration varied from two minutes to one day. Four patients had brain tumors, six probably had brain infarcts, and one a degenerative process. The differential diagnosis included TIAs, migraine accompaniments, and seizures. In the absence of good evidence for the first two, the cases are discussed from the standpoint of possibly representing nonconvulsive seizure paralysis (ictal paralysis, inhibitory seizure paralysis or somatic inhibitory seizure). Because of the difficulty in defining seizures as well as TIAs and migraine in their atypical variations, a firm conclusion concerning the mechanisms of the spells was not attained. Two cases of the hypertensive amaurosis-seizure syndrome have been added as further examples of ictal deficits.
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2/47. Postoperative pseudostatus: not everything that shakes is epilepsy.

    Postoperative epileptic seizures are recognised but rare. Psychogenic seizures and pseudostatus epilepticus are relatively common, particularly in the peri-operative period. Our series of five cases of postoperative pseudostatus epilepticus demonstrates that the failure to recognise the psychogenic nature of this condition may cause anaesthetists to give inappropriate and potentially harmful treatment. Psychogenic 'status' is easy to diagnose once it has been considered. Convulsive episodes lasting longer than 90 s, closed eyes during a 'tonic-clonic' attack, retained pupillary response and resistance to eye opening are useful signs. Often there is a history of multiple admissions with 'status epilepticus' and of previous postoperative 'status'.
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3/47. Convulsive-like movements in brainstem stroke.

    BACKGROUND: Involuntary convulsive-like movements sometimes occur in patients with brainstem strokes. These movements vary in nature, frequency, and trigger, including fasciculation-like, shivering, jerky, tonic-clonic, and intermittent shaking movements. Some are interpreted as decerebrate postures or seizures. It is important to recognize this type of motor phenomenon since it may be a diagnostic clue for early diagnosis and treatment of brainstem strokes. DESIGN: Case report and review of the literature observation: A 72-year-old-man presented with impaired consciousness and jerks of the upper limbs mimicking seizures. These episodes consisted of brief clonic contractions of the proximal and distal upper extremities. They were observed in paroxysms lasting for 3 to 5 seconds. magnetic resonance imaging showed large midpontine infarction. magnetic resonance angiography revealed the absence of basilar artery blood flow. No seizure discharges were observed in the electroencephalogram. Anticoagulation with intravenous heparin was started. Two days after admission, the patient had a cardiac arrest and died. We review the frequency and nature of convulsive-like movements in brainstem stroke in the literature. CONCLUSIONS: Movements associated with brainstem lesions are not easily differentiated from convulsions. Unexpected onset and inexperience of the observers limit the characterization of this phenomenon. Convulsive-like movements in brainstem stroke may occur more frequently than reported. Early detection of this motor phenomenon may have practical implications.
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4/47. Management of the difficult patient with complex partial seizures.

    Successful treatment of partial complex seizures (and the epilepsies in general) is a process of management over time and involves several factors. It starts with accurate and adequate diagnostic formulations. To this end the physician must be thoroughly familiar with the ictal manifestations of partial complex and other epilepsies as well as the clinical features of other transient but reversible episodes that might present difficulties in differential diagnosis. The diagnosis is based upon the history of a patient experiencing partial complex seizures, and the most important resource is a careful and detailed history of the ictal events and the circumstances under which they occur. Clinical observation and electrophysiologic monitoring of the patient during attacks, either spontaneous or induced, is the most powerful technique available in cases which present difficult diagnostic problems. In addition to recognition and proper classification of the seizures themselves, diagnosis and treatment of the cause of the seizures, particularly when it is an active disease, is of prime importance. Even after accurate diagnosis, the heterogenous nature of the population of patients with partial complex seizures is such that marked variation in response to treatment with antiepileptic drugs is to be anticipated. The use of these drugs must be individualized and based upon a thorough and working knowledge of their clinical pharmacology. The most frequent mistakes in our experience have been prescribing the drugs in too little doses or for too short a time. Less often the problem is overmedication. The best indicator of the effectiveness of the drugs is the clinical response of the individual patient, and in general each drug should be prescribed in increasing doses until either the seizures are controlled or unacceptable degrees of toxicity develop. The use of serum level determinations can be very helpful if not invaluable, particularly in identifying and understanding potential adverse effects of the drugs. Patient noncompliance in adhering to drug schedules is widespread, but usually can be detected by measuring serum levels. Even with the most efficient use of the drugs, however, some patients will be intractable, and elective surgical treatment should be considered. Finally, control of seizure occurrence alone is not necessarily adequate treatment, as many patients will have difficult psychosocial problems associated with their epilepsy. Treatment of such associated problems is necessary on its own merits, but occasionally can result in significant improvement in seizure control.
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5/47. Schinzel-Giedion syndrome: a further cause of West syndrome.

    Schinzel-Giedion syndrome (SGS) is a rare disorder with a likely autosomal recessive pattern of inheritance which is characterized by several facial dysmorphisms, midface hypoplasia, multiple skeletal anomalies including short and sclerotic skull base, short neck, and post-axial polydactyly. Cardiac and urogenital malformations are also present. Thirty-three cases have been described so far. We report on a boy affected by SGS in whom a long-term EEG follow-up showed a progressive deterioration of the background bioelectric activity ending, at the age of 19 months, with a hypsarrhythmic pattern clinically correlated with severe and refractory infantile spasms. EEG deterioration and neuroradiological findings, which showed progressive brain atrophy, confirm the neurodegenerative nature of SGS. We also re-evaluated all the published cases and found that 33% of patients with SGS experienced neonatal seizures and another 25% developed West syndrome in the following months. The seizures appeared extremely refractory to several anticonvulsive treatments. In conclusion, we believe that SGS should be included among the causes of secondary West syndrome.
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6/47. MELAS: clinical phenotype and morphological brain abnormalities.

    We describe the clinical and neuropathological findings of three unrelated autopsy cases of MELAS harboring the A3243G transition in the mitochondrial dna (mtDNA). Using immunohistochemical techniques, we studied the expression of several subunits of the respiratory chain in various brain regions from the same cases. In all three cases there was a reduced immunocytochemical staining for mtDNA-encoded subunits of the respiratory chain, confirming the presence of a defective mitochondrial protein synthesis in this disease. Mitochondrial abnormalities were mostly confined to multiple areas of different size and shape, in agreement with the focal character of the brain pathology in MELAS, and were most prominent in the cerebral cortex, providing a morphological contribution to the explanation of the cognitive regression of the patients. Immunoreactivity for mtDNA-encoded subunits was reduced in the walls of many pial and intracerebral arterioles of different brain regions but there was no clear correlation between territories of affected vessels and distribution of the histological and immunohistochemical lesions. Cerebral focal lesions in MELAS might have a metabolic nature and several pathogenetic mechanisms might be involved in the genesis of stroke-like episodes when there is a local increased ATP demand.
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7/47. Reversible postictal MRI change mimicking structural lesion.

    A reversible change on magnetic resonance imaging (MRI) following generalised seizure mimicking a tumour-like structural lesion is reported in a 15-year-old patient. MRI revealed a left fronto-parietal cortico-subcortical lesion on T2 weighted images. The control MRI after 5 weeks from the onset revealed no pathological finding. The reversible MRI changes may be the result of a local brain swelling, and a defect of cerebral autoregulation during seizure at the site of activity. The transient nature of such neuroradiological findings have to be taken into consideration in the differential diagnosis because of their similar appearance on imaging to intrinsic brain tumours.
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8/47. Epileptic fits or infantile masturbation?

    Two infants, one girl, 5 months old, and one boy, 6 months old, presented with rhythmic and sustained motor activities of a stereotyped nature accompanied by moaning and grunting, facial flushing and altered awareness. The episodes occurred frequently and were initially believed to be epileptic. Normal electroencephalograms during the fits, lack of response to antiepileptic medication given to one child and careful reviewing of videotape recordings, enabled us eventually to diagnose the 'seizure-like' episodes as masturbatory activity.
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9/47. Group therapy in the management of epilepsy.

    A pilot project using group therapy with patients from an epilepsy clinic is described. Observations of the group show that members were able to discuss their feelings about having epilepsy, and the difficulties the disorder caused in all areas of their lives, and to tolerate and learn from seizures which took place during group meetings. The greatest benefit was meeting others with epilepsy and sharing experiences in an environment that was not overprotective or overanxious, in contrast to their usual surroundings. The author suggests that groups of a similar nature could be a useful addition to the management of people with epilepsy.
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10/47. Insights into the pathogenesis of cerebral lesions in incontinentia pigmenti.

    We report the case of a neonate with incontinentia pigmenti and seizures on day 4 of life who underwent magnetic resonance imaging and angiography scanning at 8, 13, and 21 days of age. The serial magnetic resonance images demonstrated the evolution of acute microvascular hemorrhagic infarcts in the periventricular white matter in the first week of life. The associated magnetic resonance angiogram findings consisted of decreased branching and poor filling of intracerebral vessels. This report adds important insights into the nature and timing of cerebral lesions in incontinentia pigmenti.
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