Gurendra Char

Stroke in a cohort of patients with homozygous sickle cell disease

Strokes occurred in 17 of 310 children with homozygous sickle cell disease who were followed from birth, representing an incidence of 7.8% by the age of 14 years. Two children had subarachnoid hemorrhage, one having resolution of symptoms after aneurysm surgery and another dying of a presumed second hemorrhage 14 days later. The remaining 15 strokes were presumed to be cerebral infarction, although autopsy, angiographic, or computed tomographic evidence was available in only 8 children. There were 6 deaths, 2 in the acute event and 4 after recurrence, which occurred in 6 (46%) of 13 patients who survived the initial episode. There were 10 recurrent episodes at a median interval of 9 months after the initial event. Steady-state hematologic data revealed significantly higher leukocyte counts than in control subjects without strokes at age 1 year and in the last study preceding the stroke. The initial stroke coincided with an acutely lowered hemoglobin value in 5 patients (3 aplastic crises, 1 acute splenic sequestration, 1 probable pulmonary sequestration) and with painful crises in another 7 patients. We conclude that a high leukocyte count and an acute decrease of hemoglobin are risk factors for stroke in patients with homozygous sickle cell disease.

HTLV-1 and polymyositis in Jamaica

IgG antibodies to human T-cell lymphotropic virus (HTLV-1) were found in 11 of 13 (85%) Jamaican patients with idiopathic adult polymyositis. The association was first observed in 7 patients with polymyositis who were included in a control group of 100 patients with neurological and neuromuscular diseases in a serological investigation of the prevalence of HTLV-1 antibody in patients with tropical spastic paraparesis. All 7 patients with polymyositis were positive for the antibody by an enzyme-linked immunosorbent assay, confirmed by western blot. Because of this striking association a further 6 patients with polymyositis were identified and tested, 4 of whom were also seropositive for HTLV-1 antibody.

Postmenopausal uterine inversion treated by subtotal hysterectomy

Uterine inversion is extremely rare as a gynaecological complication with the majority of cases being reported as puerperal in the reproductive age group after a vaginal delivery. Non-puerperal cases are difficult to diagnose and most cases are diagnosed at surgery. Most of these reported cases are associated with uterine pathology, such as uterine fibroids, endometrial polyps, sarcomas or endometrial carcinomas. We report a case associated with multiple uterine pathology.

Intravenous leiomyomatosis with massive ascites

A case of intravenous leiomyomatosis with massive ascites is reported. This is the first such recorded case. The patient was treated with a subtotal abdominal hysterectomy and bilateral salpingo‐oophorectomy. Pathological examination established a vessel wall origin. There is no evidence of recurrence up to 20 months after initial treatment.

Chronic inflammatory demyelinating polyneuropathy in a patient infected with human T lymphotropic virus type I.

A 32-year-old Afro-Caribbean woman presented with a 1-year history of slowly progressive sensory and motor symptoms initially affecting the legs and later involving the arms. Clinical examination demonstrated a mainly distal pattern of weakness with little objective sensory impairment. The clinical features suggested the possibility of chronic inflammatory demyelinating polyneuropathy. This diagnosis was supported by neurophysiological testing and examination of the cerebrospinal fluid and confirmed by sural nerve biopsy as well as by exclusion of other causes of neuropathy. Seropositivity for human T lymphotropic virus type I (HTLV-I) was demonstrated. The clinical significance of this finding in an area with a high HTLV-I endemicity, as well as its possible aetiological relevance to the diagnosis of chronic inflammatory demyelinating polyneuropathy, is discussed.