Bálint症状群を呈したCreutzfeldt-Jakob病（亜急性海綿状脳症）の1例

A CASE OF CREUTZFELDT-JAKOB DISEASE WITH BÁLINT'S SYNDROME

I．はじめに

　われわれは，臨床的に定型的なBálint症状群を，経過中に認めた亜急性海綿状脳症（Subacute SpongiformEncephalopathy）の1症例を経験した。

　Creutzfeldt-Jakob病（亜急性海綿状脳症），とくに，Heidenhain型において，後頭，頭頂葉病変にもとつく皮質性視覚障碍を認めることがある。これらの多くは，皮質盲，視覚失認であるが，定型的なBálint症状群が記載されている例はない。これは，Creutzfeldt-Jakob病のような，進行性の亜急性の経過をとる疾患では痴呆におおわれて，神経心理的な検査がなされる時期が限られていて見逃されやすいことによるところが大きいと思われる。

An autopsy case of the Creutzfeldt-Jakob disease(subacute spongiform encephalopathy) with typicalBalint's syndrome is reported in the present paper.

A 55-year-old woman was admitted to the HyogoMedical College Hospital in paranoid-hallucinatorystate with marked dementia.

On admission, psychiatric examination revealedan amnestic syndrome with disorientation for timeand place, visuo-constructive disturbance, confabula-tion, metamorphopsia and dressing apraxia.

No neurological abnormalities were noted. La-boratory investigations revealed no abnormalities.

Five months after the onset of the illness, typical Balint's syndrome, i. e. psychic paralysis of the visualfixation, optic ataxia and disturbance of attentionwere noted. After the admission to the hospital,mental deterioration with echosymptomes, myoclonicjerks, and paratonic rigidity (Gegenhalten) weregradually developed and the patient was bedriddenwith the abnormal posture (quadriplegia in flexion).

Dysphagia and urinary incontinence was alsoseen in this stage. Three months later, she diedof dyspnea, due to the respiratory infection andepileptic status. The duration of the illness was8 months.

Neuropathologically, spongy degeneration andastrocytosis were seen in the cerebral cortex, notablyin the temporo-occipital and front-basal regions.Marked gemistocytic proliferation and slight states(état grumeleaux) were noted in the calcarine areasof both hemispheres. Proliferation of protoplasmicastrocytes were found in the severely affected thal-amic regions, such as lateral and medial thalamicnuclei. Medial geniculate bodies showed a markedneuronal degeneration. Bálint's syndrome in theCreutzfeldt-Jakob's disease was discussed briefly inthis paper.