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

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

The simultaneous appearance of congenital infiltrating lipomatosis of the face that causes facial hemihypertrophy and ipsilateral hemimegalencephaly is extremely rare. We report a 4-year-old boy with congenital facial asymmetry and infantile-onset epilepsy. Magnetic resonance imaging (MRI) results led to the diagnosis of infiltrating lipomatosis of the face; the diagnosis was confirmed on the basis of the results of pathological examinations. Additionally, brain MRI revealed ipsilateral hemimegalencephaly, associated with band heterotopia and the hemihypertrophy of the ipsilateral brainstem and cerebellum. He had no nevi or other skin abnormalities suggesting neurocutaneous syndrome. His seizures were so intractable that they necessitated functional hemispherectomy. The lipomatous lesion was successfully resected without relapse. Psychomotor delay and left hemiplegia were observed at the last follow-up.
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PMID:Congenital infiltrating lipomatosis of the face with ipsilateral hemimegalencephaly, band heterotopia, and hypertrophy of brainstem and cerebellum. 2085 36