Biomedical subjects
Publications and source records attributed to R G Curless.
Two brothers are described with the previously unrecognized combination of Leber amaurosis and abnormal myelin detected by magnetic resonance imaging. Both have evidence of delayed psychomotor development and one has autistic features. A possible relationship with infantile autism or a peroxisomal dysfunction syndrome is explored. No peroxisomal defect was found in these patients.
An 18-year-old Hispanic female developed symptoms of a Histoplasma capsulatum infection at 9 1/2 years of age. Initial problems of ataxia and hydrocephalus were followed by seizures and a cranial nerve palsy which developed over several years. T1-weighted cranial magnetic resonance imaging studies revealed numerous high-signal lesions; subsequently, signs of multisystem dissemination developed. Treatment included ventriculoperitoneal shunting, corticosteroids, amphotericin B, and a new antifungal agent, fluconazole, which was responsible for marked improvement.
Band heterotopia is a severe form of neuronal migration disorder associated with intractable epilepsy and neurologic impairment. Surgical treatment of seizures associated with this malformation has not been reported previously. We report a patient with band heterotopia and poorly controlled atonic seizures causing falls and injury. The patient was treated with anterior corpus callosotomy, with significant postoperative decrease in seizure frequency. Corpus callosotomy is a reasonable alternative to consider in management of patients with cortical heterotopia and intractable seizures.
Serial CT findings in an infant with HTLV-III infection and cytomegalic inclusion virus encephalitis are presented.