Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three types of clinical features at the onset are well known to be characteristic of acute hemiplegia syndrome (AHS). Type 1 comprises status epilepticus of hemiconvulsions with fever. Representative diseases of this type are the infectious diseases of the central nervous system, acute encephalopathy and cerebral vascular diseases. Type 2 comprises status epilepticus of hemiconvulsions without fever. Cerebral vascular diseases and epilepsy are the major ones of this type. Type 3 comprises hemiplegia or hemiparesis of sudden onset without fever or convulsions. Most patients with this type had cerebral vascular diseases, about half of which were moyamoya disease in Japan. Recent progress in neuroimaging studies has allowed considerable elucidation of the etiology of AHS. Gadolinium-enhanced MRI showed minimal lesions such as capsular infarction more clearly than plain MRI. Acetazolamide test 99mTc-HMPAO SPECT imaging is one of the useful assisted diagnostic techniques for moyamoya disease, because it reveals the reserve capacity of the collaterals. [123I]IMP SPECT is useful for the diagnosis and follow-up of acute disseminated encephalomyelitis (ADEM), as the images of the lesions coincide well with the MRI ones. 99mTc-HMPAO SPECT in a case with alternating hemiplegia revealed normoperfusion in the ictal periods. Four cases of AHS are reported here.
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PMID:Acute hemiplegia syndrome in childhood. 805 23

Hemimegalencephaly is a rare disorder manifest by early epilepsy, mental retardation and hemiplegia. The neuropathology has been described in only 15 cases to date. The present case provides a further description of the pathology in a hemispherectomy specimen and shows unusual features, including cystic breakdown of the white matter possibly related to the long duration of the features, including cystic breakdown of the white matter possibly related to the long duration of the disease; the subject was 13 years older than previously documented cases. MRI findings were also unusual in showing mass effect and ventricular compression in the affected hemisphere, features not previously described in hemimegalencephaly.
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PMID:Unusual magnetic resonance and neuropathological findings in hemimegalencephaly: report of a case following hemispherectomy. 815 58

Two cases are described which presented with diffuse swelling of one cheek and hypertrophy of the underlying maxilla and mandible. Both children developed verrucous pigmented streaks over the area of swelling, and had epilepsy and severe mental subnormality. One of the children had a contralateral hemiplegia and his condition had a progressive course. The other child had no focal neurological signs and his disease seemed to be non-progressive. Although the facial appearance of both children suggested the diagnosis of encephalocrainiocutaneous lipomatosis syndrome, it was not possible to demonstrate the presence of any lipomata in either case. The literature relating to encephalocraniocutaneous lipomatosis is reviewed, as well as that relating to the syndromes of naevus unius lateris, the Proteus syndrome and the syndrome of cranial hemihypertrophy, and the clinical features of our two cases are compared with the features of these four syndromes.
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PMID:Cranial hemihypertrophy with ipsilateral naevoid streaks, intellectual handicap and epilepsy: a report of two cases. 831 22

We report 22 cases of alternating hemiplegia of childhood. In addition to repeated episodes of hemiplegia lasting from a few minutes to several days, the disease was characterized by an onset before 18 months of age, the occurrence of tonic or dystonic attacks, nystagmus, dyspnea and other autonomic phenomena, and the development of cognitive impairment and of a choreoathetotic movement disorder. All the patients also had episodes of quadriplegia that occurred either when a hemiplegia was shifting from one side to the other or as an isolated manifestation. Such episodes were often severe and followed by developmental deterioration. In all children, sleep consistently relieved both weakness and associated paroxysmal phenomena, but these would reappear 10 to 20 minutes after the children awakened, during long-lasting episodes. Although six patients also had epileptic seizures, the condition seems to be distinct from epilepsy, and the clinical features and poor outcome differentiate it from migraine. Treatment with the calcium-entry blocker flunarizine was partially effective.
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PMID:Alternating hemiplegia of childhood. 849 42

Electroencephalography (EEG) was performed in 13 male patients with cerebral malaria during the first 24 hours of admission, using a 10-channel, 10-20 system EEG machine (6 montages, 20 minute duration). The EEG patterns were of theta and delta waves from both sides of cerebral hemisphere suggesting diffused cortical dysfunction. No epileptic pattern was found in patients who had seizures prior to, or after admission. The initial EEG performed on the day of admission did not show any specific pattern attributable to any pathological condition. It was also unable to predict the prognosis of the 2 dead patients. However, one cerebral malaria patient with left hemiplegia was subsequently found to have right basal ganglia hemorrhage in CAT scan, high amplitude delta waves and theta waves in the tracings of the right hemisphere. The study suggests that a single EEG data on admission can hardly give enough information for prediction of the clinical course and outcome of cerebral malaria. Serial EEGs probably provide more useful information regarding the prognostic signs in this group of patients. Nevertheless, EEG could be useful to rule out some cerebral pathology such as space occupying lesions, epilepsy or any other causes of unconsciousness that could produce similar cerebral symptoms in malaria patients.
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PMID:Electroencephalography in cerebral malaria. 852 17

Sixty-eight children with malignant brain tumors were treated with the "8 in 1" chemotherapy protocol from 1986 to 1993 in Finland. The overall 5-year survival rate was 43%. Thirty-one children are still alive and tumor-free, and have been evaluated in the present study. Of these 31 children, 26% had hemi- or tetraplegia, 13% intractable seizures, and 30% attend special schools. The mean full scale (FS) IQ was 85 (range 45-138), 24% had an FSIQ value less than 70, and 36% more than 90. One-half of the survivors were placed in Bloom's group I or II, are able to lead an active life, and have only mild neurologic disabilities. In the other, neurologic late complications accumulated and these children were relegated to Bloom's group III or IV, with major disabilities such as hemiplegia, intractable epilepsy, or mental retardation. The most important prognostic factors were severe perioperative complications, young age at diagnosis, and cranial irradiation.
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PMID:Neuropsychologic late effects in children with malignant brain tumors treated with surgery, radiotherapy and "8 in 1" chemotherapy. 883 71

Fourteen children with spastic hemiplegia of various etiology: cerebral palsy (seven cases, five with porencephalic cyst); stroke with small deep infarcts (two); hemiconvulsion-hemiplegia-epilepsy syndrome (three); traumatic brain injury (two), were investigated by 99mTc-HMPAO SPECT. Localized and remote perfusion abnormalities were studied. Hypoperfusion corresponding to CT abnormalities was found in each group, but the perfusion deficit extended beyond the boundaries of anatomical defects, most prominently in cases with widespread unilateral epileptic discharges. Ipsilateral cerebellar diaschisis was observed in patients with early cerebral insult (who had porencephalic cyst of pre- or perinatal onset) and crossed cerebellar diaschisis was noted in a patient who sustained traumatic brain injury at a later age. Diaschisis in the overlying cortex, thalamus and basal ganglia was noticed in several cases. Although clinical symptoms or signs could not be unequivocally attributed either to the size of the perfusion defects beyond the boundaries of lesions shown by CT or to the diaschisis, the findings may contribute to reveal age-related abnormal perfusion patterns.
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PMID:Regional cerebral blood perfusion in children with hemiplegia: A SPECT study. 889 65

We described a profoundly intellectually disabled 24-year-old man with Wolf-Hirschhorn syndrome, left hemiplegia, epilepsy, atrophy of the right cerebral hemisphere, and dilatation of the right ventricle. The patient had a small ventricular septal defect, was wheelchair bound, and totally dependent. He had no speech, but vocalised to show his feelings. In this patient, the del(4)(p15) was subtle and arose due to the inheritance of a recombinant chromosome (4) from a maternal pericentric inversion-46,XX,inv(4) (p15.32q35). Fluorescence in situ hybridisation with probe D4S96 confirmed the deletion. This is the second case of Wolf-Hirschhorn syndrome resulting from a large pericentric inversion of chromosome 4.
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PMID:The Wolf-Hirschhorn syndrome in adulthood: evaluation of a 24-year-old man with a rec(4) chromosome. 891 3

Two forms of unilateral tonic (hemitonic) seizure were observed in two adult epileptic patients with infantile hemiplegia. Their ictal EEGs showed diffuse bilaterally synchronous spike bursts without focal features. In one case, a 31-year-old male, the VTR/EEG recording clearly demonstrated hemitonic seizure which simultaneously involved his paretic limbs and body and were associated with loss of consciousness and autonomic signs. In the other case, a 33-year-old male, partial seizures with secondary unilateral generalization were demonstrated by VTR/EEG recording. The reason why these cases do not exhibit motor seizure activity on the unaffected side despite diffuse bilateral EEG changes, a lower motor threshold in the paretic limbs and inhibitory effect in the brain-stem on undamaged side are postulated. These two cases suggest that hemitonic seizures include heterogeneous characteristics of partial and generalized epilepsy. In patients with diffuse brain damage, such as those with infantile hemiplegia, seizures appear to exhibit complicated patterns, with characteristics of partial and generalized seizures. There is a gradiation of expression of unilateral seizure, range from clearly partial seizures to those with mostly features of generalized seizures, except for an asymmetry of motor expression.
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PMID:Two forms of hemitonic seizure in patients with infantile hemiplegia and epilepsy. 906 29

Ninety eight children with epilepsy attending the Neurology clinic, University of Ilorin Teaching Hospital over a two year period were studied prospectively. Males were more affected than females in a ratio of 5:3. Generalised tonic-clonic seizures accounted for 62.2% of the cases, and partial seizures for 17.4%. Infantile spasms were seen exclusively in infants less than two years old and absence and generalised seizures in children more than three years of age. Skull radiography showed abnormal findings in 11.2%. Ectroencephalography showed typical findings in 43.9%. Hemiplegia was the most common neurological sequelae (30.3%). Other sequelae include hyperactivity, irrational behaviour, expressive aphasia, mental subnormality, deafness, and blindness in that order. Therapy with a single appropriate anticonvulsant was usually effective for seizure control except in some patients with focal seizures, infantile spasms, severely delayed developmental milestones and prolonged seizures. Poor drug compliance remains the major constraint to adequate seizure control, further compounded in this environment by nonavailability of drugs and unaffordable costs.
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PMID:Childhood epilepsy in Ilorin, Nigeria. 918 88


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