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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report the brain findings in six cases of familial erythrophagocytic lymphohistiocytosis (FEL) and review the literature, focusing primarily on the neurologic and neuropathologic aspects. Clinically, the most common neurologic abnormalities in the six patients were stupor or coma and seizures, with hemiplegia and cranial nerve palsies. Neuropathologic changes were characterized by infiltration of the meninges by lymphocytes and histiocytes and perivascular lymphocytic cuffing and diffuse proliferation of histiocytes in the brain parenchyma. The severity of the brain involvement was variable. In the mildest case there was only meningeal involvement. More severely involved cases showed meningeal and perivascular infiltration of lymphoid cells in the brain. In the most severely involved cases, there was, in addition, diffuse cerebral infiltration by histiocytes accompanied by multifocal necrosis. Some cases showed disproportionately greater involvement of the brain than of the visceral organs. The authors conclude that "meningoencephalitis," histiocytic invasion, and necrotizing brain lesions are characteristic of FEL, although the mechanism of their production is not known. Frequent seizures and disseminated intravascular coagulopathy may be contributory.
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PMID:Neuropathology of familial erythrophagocytic lymphohistiocytosis: six cases and review of the literature. 669 35

We describe a family in which two males and seven females have brown pigmentation of the skin. In the females, the type and distribution of the pigmentation mimicked incontinentia pigmenti; in the males, the pattern was reticulate. The histological appearance was the same in both sexes with amyloid deposits in the papillary dermis, melanin in the basal layer, and slight hyperkeratosis. The females were otherwise normal. Both males had thrived poorly as infants but had survived. One had severe gastroenteritis with blood in the stools starting at the age of three weeks followed by seizures, hemiplegia, and developmental delay; the other had recurrent pneumonia throughout life, a urethral stricture, inguinal herniae, and near-blindness from amyloid deposition in the cornea. Five other males in the family had had severe illnesses. Two died of pneumonia by three months. One died at three months from colitis. Both remaining boys had colitis as infants, failed to thrive, and developed recurrent pneumonia from which one died at three years. We think all of these relatives had the same disease carried by a single gene with pleiotropic effects. The most likely form of inheritance is X-linked.
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PMID:Familial cutaneous amyloidosis with systemic manifestations in males. 679 69

Fifteen children between the age of 3 and 7 years who had had unilateral seizure followed by a transitory hemiplegia (without fever) were observed. This seizure was the only one in 13 of the children; a second fit appeared in 2 others. The average duration of the observation period was 10 years, 6 months. The evolution of the EEG shows that after a post-ictal delta focus contralateral to the hemiplegia, spike foci of variable localisations were formed in 12 cases. These cases, chosen because of their benign evolution, seem to be related closely to rolandic paroxysmal epilepsy (EPR).
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PMID:[Unilateral epilepsy with hemiplegia in the child (author's transl)]. 680 2

Twenty three patients are described with a Kojewnikow syndrome in order to identify possible etiological factors. According to their history, clinical and E.E.G. features, patients could be classified into two groups; 1) Eleven patients corresponded to the classical description of the Kojewnikow syndrome: variable age at onset of the disease, rare somatomotor seizures, delay of onset of myoclonic jerks often of long duration, myoclonus limited to a small region, normal neurological examination, stable hemiplegia, normal results of psychometric tests, localised E.E.G. alterations. In most of these patients the etiology of the disorder was known, and the symptoms could be related to a localised lesion of the central cortex; 2) the second group (11 patients) presented with completely different characteristics: early onset of fits (age: 2 to 10 years), presence of other seizure types, short delay of onset of myoclonic jerks, high frequency of seizures, localization of myoclonus over large parts of the body, progressive evolution of a motor syndrome, associated neurological signs and disorders of sleep and behavior, progressive mental deterioration, characteristic E.E.G. pattern with long subclinical paroxysms of slow spikes (sharp waves) with variable localization, diffuse cerebral lesions of unknown etiology were found. Only one patient could not be allocated to either group. Several circumstantial data suggest that patients of the second group may suffer from a slow virus infection. Should this hypothesis be confirmed, a reconsideration of the Kojewnikow syndrome from the etiological standpoint would be of great theoretical and practical interest.
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PMID:[Continuous partial epilepsy: syndrome and disease]. 682 Jan 77

The serious, late complication of superficial cerebral hemosiderosis, which appears after several years in 1/4-1/3 of patients who have undergone hemispherectomy, has resulted in recent years in a considerable reluctance to carry out this operation despite the fact it has proved to be highly effective in patients with medically refractory seizures associated with hemiplegia. Preservation of a small portion of the hemisphere, usually the frontal or occipital pole, has proved to be effective in preventing this late complication, but at the cost of a significant reduction in the effectiveness of the operation in reducing the patients' seizure tendency. Preserving the frontal and occipital poles but disconnecting them from the rest of the brain, resulting in a functional complete but anatomical subtotal hemispherectomy, retains the therapeutic effectiveness of a complete hemispherectomy while still protecting adequately against the serious late postoperative complication of superficial cerebral hemosiderosis and its associated neurologic deterioration, hydrocephalus and sometimes death.
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PMID:Hemispherectomy for seizures revisited. 686 Oct 11

Two patients with epilepsy and large hemispheric lesions underwent section of the frontal fibres of the corpus callosum for the treatment of seizures refractory to medical treatment. A severely retarded girl of 18 had encephalotrigeminal angiomatosis (Sturge-Weber syndrome) with multiple daily absences, tonic-clonic, myoclonic, atonic and adversive seizures since infancy. All types of fits--with the exception of adversive seizures and rare tonic-clonic fits--disappeared after anterior callosotomy. Another moderately retarded girl of 18 had an old cystic lesion over the entire territory of the left middle cerebral artery. She had had right hemiplegia since infancy and frequent brief absences and massive myoclonus triggered by unexpected sensory stimuli since the age of six years. Following anterior callosotomy there was an almost complete disappearance of the absences and a marked reduction of her startle myoclonus. Frontal callosotomy is a useful procedure in epileptics with large hemispheric lesions and carries less risk than hemispherectomy or total commissurotomy.
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PMID:Anterior callosotomy as a substitute for hemispherectomy. 693 7

Children presenting after trauma with headache, seizures, hemiplegia and coma may have an intracranial dissecting aneurysm. Specific angiographic findings provide confirmation of this diagnosis. The dissection occurs subintimally and differs clinically and pathologically from dissecting aneurysms of extracranial arteries. The course in children beyond infancy is catastrophic, justifying consideration of potentially life saving surgical intervention.
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PMID:Intracranial dissecting aneurysms in childhood. 706 92

Lipoma of the corpus callosum is a rare intracranial condition, possibly congenital. It is often asymptomatic, but may present with headaches, seizures, hemiplegia, or dementia. An example of this disease is reported, and the condition reviewed. The radiological appearance is described, with emphasis on the value of computerized tomography in making the diagnosis. Surgery is of limited value but may be required in specially selected cases.
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PMID:Lipoma of the corpus callosum. Case report. 708 24

Eighty-three children with spastic cerebral palsy (CP) were examined with cranial CT. In 56 cases the CT findings were abnormal. The most frequent abnormality was atrophy, present in 44 patients. The frequency of pathologic CT increased with severity of the CP. Patients with CP of postnatal aetiology more often had abnormal CT than patients with other known causes. Pathologic CT findings were seen more often in patients with seizures than in patients without. Infarctions and hemiatrophy were much more frequent in patients with hemiplegia than in patients with other types of spastic CP. A special kind of central atrophy, called isolated atrophy around in the cella media, is described. This condition was seen in 20% of cases, most often in hemi- and paraplegic patients. Early infarctions in the border areas between the vascular territories of the internal carotid and the posterior cerebral artery may be the reason for this kind of atrophy.
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PMID:Computed tomography in spastic cerebral palsy. 712 22

Of 27 patients with hypoplastic anemia treated between 1971 and 1974 with male hormone and protein-assimilating hormone, 3 developed superior sagittal sinus thrombosis (SSST). The clinical symptoms and signs and angiographic findings of SST were characteristic enough to allow an early diagnosis. Signs related to SST were seizures, hemiplegia, facial palsy, stupor, and coma, with the most important prodrome and consistent subjective complaint being headache. Following discontinuation of the hormone therapy, neurological signs and symptoms related to SSST gradually subsided. In all cases, the hematological picture improved with discontinuation of the hormone therapies. It appears that administration of male hormone can be associated with the development of SSST. If neurological symptoms and signs of SSST appear, administration of the hormones should be discontinued.
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PMID:Superior sagittal sinus thrombosis associated with androgen therapy for hypoplastic anemia. 715 62


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