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)

We have reviewed 50 cases of Wegener's granulomatosis, seen at the New England Medical Center Hospital between 1970 and 1984, and were impressed that 10 (20%) of these patients were under 25 years of age, with ages ranging from 13 to 23 years. Closer examination of this younger group revealed striking differences in their presenting symptoms and organ involvement when compared to the older group of patients. The presentation of these young patients was varied, with no single predominant symptom. Patients presented with otalgia and otitis media or hearing loss, fulminant sinusitis, arthralgias, and even corneal ulcers. Only one patient had "typical" rhinitis and nasal congestion. This group also had a disproportionate number of patients with involvement of the oral cavity, skin, and trachea. Biopsy of these sites frequently demonstrated necrotizing vasculitis. Three of our 50 patients had intracranial involvement, leading to transient hemiplegia in the first, permanent hemiplegia in the second, and a seizure disorder in the third. Two of these patients were in the younger age group. The proportion of patients with limited and generalized Wegener's granulomatosis was the same in both the younger and older age groups. All the younger patients, however, had manifestations of the disease in the head and neck, while four of the older patients had no symptoms in the upper respiratory tract. The number of young patients in our study emphasizes the fact that Wegener's granulomatosis, indeed, occurs in the younger patient and with a greater frequency than previously supposed. This study suggests that in the teenager and young adult, with an unusual constellation of symptoms of the head and neck and accompanying systemic problems, a diagnosis of Wegener's granulomatosis should be seriously considered.
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PMID:Presentation of Wegener's granulomatosis in young patients. 308 67

A 20-year-old woman with myelomeningocele presented with acute right-ear pain and right hemiplegia which improved, but then progressively deteriorated. Surgery, after MRI, revealed a large arteriovenous malformation (AVM) involving the right side of the upper cervical cord and brainstem. The woman also had two epidermal nevi at the same site as the spinal cord AVMs. There has been no previous report of an association of myelomeningocele, spinal cord AVMs and epidermal nevi syndrome. The same location of the nevus and spinal cord AVMs, with a proposed common pathogenesis, raise the possibility that the association may be more than chance occurrence. Spinal cord AVMs should be considered in patients with myelomeningocele and similar clinical features.
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PMID:Myelomeningocele, spinal arteriovenous malformations and epidermal nevi syndrome: a possible rare association? 833 63