An autopsy case of myotonic dystrophy with mental disorders and various neuropathologic features.

An autopsy case of myotonic dystrophy (MD) is reported. The patient was a 58-year-old male. He presented with muscular weakness and muscular atrophy at the age of 33 and was diagnosed as having MD from myotonic symptoms (i.e. percussion and grip myotonia) at 49 years old. Mental disorders including a delusional hallucinatory state, mental slowness, indifference, and lack of spontaneity as well as visual cognitive impairments were noted at the age of 55. He showed Parkinsonism and died of septic shock. T2-weighted magnetic resonance imaging demonstrated diffuse cortical atrophy with a marked frontal atrophy and high-intensity signals in the white matter. Single photon emission computed tomography demonstrated hypoperfusion in the frontal cortex. Neuropathologic observation revealed neuronal loss in the superficial layer of the frontal and parietal cortices and extensive neuronal loss in the occipital cortex, intracytoplasmic inclusion body in the nerve cell of the medial thalamic nuclei, neuronal loss and presence of Lewy bodies in the substantia nigra and locus ceruleus corresponding to the pathologic features of Parkinson's disease, as well as abnormalities of myelin in the white matter. The present case suggests that in MD brain, various neuropathologic changes may occur and they contribute to the mental disorders.

CT, MR, and SPECT findings in a general paresis.

CNS changes in a case with general paresis were investigated by X-ray computed tomography (CT), Magnetic resonance (MR), and single photon emission computed tomography (SPECT). CT and MR showed a mild degree of diffuse cortical atrophy and the dilatation of lateral ventricles with no signs of ischemic lesions or inflammations. On the other hand, SPECT using 123I-N-isopropyl-p-iodoamphetamine (123I-IMP demonstrated marked reduction of the cerebral blood flow especially in the bilateral frontal and temporal cortices. Moreover, the reduction of the blood flow was significantly improved after the antisyphilitic therapy, correlated with the improvement of the mental disorders. These observations suggested that the SPECT is a useful method to evaluate the brain dysfunctions, and to assess the effect of antisyphilitic therapy in the patients with general paresis.

An autopsied case of acute lymphocytic meningoencephalitis with a long clinical course: special reference to CT, MR, SPECT and neuropathological findings.

This report describes the clinico-pathological features of acute lymphocytic meningoencephalitis (ALME) of a patient with a long clinical course of 24 months. The patient rapidly developed a confusional state, occasionally with stupor, various involuntary movements and generalized convulsions followed by fever and headache at the age of 29. Clinical symptoms, except fever and convulsions, had not distinctly improved throughout the clinical course, and cortical atrophy on CT and MR gradually progressed. SPECT revealed a low blood perfusion in the cerebral cortices. Neuropathologically, inflammatory findings were very mild, but nerve cells degenerated prominently. From these clinicopathological findings, it is suggested that chronic degenerative changes followed an acute inflammatory phase.