SERIAL MAGNETIC-RESONANCE IMAGES IN A PATIENT WITH CONGENITAL SENSORYNEUROPATHY WITH ANHIDROSIS AND COMPLICATIONS RESEMBLING HEAT-STROKE

We report the results of serial computerized tomography (CT) and magne tic resonance imaging (MRI) in a 9-month-old Japanese girl with the ra re disorder, congenital sensory neuropathy with anhidrosis (CSNA). She developed a prolonged high fever, anorexia, and weight loss with labo ratory findings of hemoconcentration and elevated levels of GOT, LDH a nd creatine phosphokinase (CK) in May 1995, and was hospitalized. The cerebrospinal fluid (CSF) was normal on admission. Elevation of CSF my elin basic protein on the 16th hospital day suggested a destruction of the myelin sheath. The first MRI performed on the 16th hospital day r evealed no marked abnormalities when the patient exhibited a high feve r, generalized tonic-clonic convulsions, and impaired consciousness. T he patient had a persistent high fever, and developed a second general ized tonic clonic convulsion and became comatose. A second MRI on the 20th hospital day showed a bilateral symmetrical paracentral hypo-inte nsity of the white matter with occipital hypo-intensity on T2-weighted images. MRI findings were considered to represent the complications o f the high fever with a loss of water from the cerebral cortices and d eep white matter. MRI and CSF findings indicated the presence of brain damage due to the high fever.