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)

There were 17 survivors of severe neonatal respiratory distress treated with IPPV for more than 24 hours (36-520 hrs, mean duration of IPPV 6 days) followed into the second year of life. 11 of these young children were physically and neurologically normal. 3 were developmentally retarded (3 months or more), 2 had neurological defects without mental subnormality. These defects (Hemiplegia and Diplegia) were correlated with low gestational ages (32 and 33 weeks respectively) 1 child was severely defective. This one was treated because of apnea caused by seizures in postmaturity syndrome (44 weeks gestation). The overall incidence of defects was 3/17 (= 17%).
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PMID:[Development after respiratortreatment during the newborn period (author's transl)]. 94 49

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

How the concept of the tuberous sclerosis complex (TSC) has developed over a period of time spanning 160 years has come form simple clinical observations, pathological studies and technological advances of imaging methods. It all began with PFO Rayer's color plate of a drawing of a patient who apparently had facial angiofibroma, published in the year 1835, and continued with von Recklinghausen's report of cardiac myomas and cerebral sclerosis in a newborn who had died minutes after birth. The seminal contribution was provided by D.M. Bourneville who, in 1880, reported and named as tuberous sclerosis the neuropathological findings in a young patient with seizures, hemiplegia, and mental subnormality who also had renal tumors. We now know that TSC is a hamartomatosis, and thanks to studies of recent years using positional cloning and DNA analysis, we are beginning to understand the biological mechanisms of these disorders which include NF1, NF2 and von Hippel-Lindau disease. Unique to TSC is that it is both phenotypically and genotypically heterogeneous. One of two suspected genes found in chromosome 16 by positional cloning has been cloned (TSC2). Another one that was discovered earlier in chromosome 9 (TSC1) has not yet been characterized. The gene product from TSC2 has been named tuberin.
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PMID:History of the tuberous sclerosis complex. 888 73

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

Myiasis is a condition caused by the invasion of tissues by larvae of Diptera flies. This phenomenon is well documented in the skin especially among animals and people in tropical and subtropical areas. The condition causes extensive tissue destruction as the larvae, at least for a certain period, feed on the host's dead or living tissue, liquid body substances, or ingested food. Mouth breathing during sleep, poor oral hygiene, alcoholism, senility, mental disability, cerebral palsy, and hemiplegia may facilitate the development of myiasis. We present a case report of oral myiasis in a 22-year-old male with cerebral palsy and severe mental retardation treated successfully by manual removal of the larvae by topical application of turpentine oil and oral systemic therapy with ivermectin.
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PMID:Oral myiasis: a case report. 2438 72