老年病変を伴ったズダン好性白質変性症の1剖検例

AN AUTOPSY CASE OF SUDANOPHILIC LEUCODYSTROPHY WITH SENILE CHANGES

はじめに

　Leucodystrophyは大脳白質に広範なびまん性脱髄巣が形成されることが特徴づけられる変性あるいは代謝障害と考えられる疾患群である。Metachromatic leuco—dystrophyのように神経化学的，酵素学的に解明されているものもあるが，sudanophilic leucodystrophy （SLD）の成因は現在なお不明である。

　今回われわれはSLDの1剖検例を経験した。病理学的に大脳白質の広範な脱髄を認め，さらに大脳皮質に老人斑を認めた。臨床的な特徴として，抑うつ状態の後に失行，失認が比較的早期にみられ，経過中にシーソー眼振，skew deviationなどの眼症状が認められた。このようなSLDの症例は報告されていないので，臨床病理学的に検討し，結果をここに報告する。

A sporadic case of sudanophilic leucodystrophy of simple form (Hallervorden) was examined clini-cally, histopathologically and neurochemically. The patient was a forty-eight year old housewife who had suffered from a depressive state for ten months at the age of thirty five. Antidepressant therapy resulted in considerable improvement of her mood and drive. Four years later, she was disoriented as to time and place. She had memory impairment, dementia, personality alterations, visual spatial agnosia, asomatognosia, constructional apraxia and dressing apraxia. See-saw nystagmus, skew devia-tion, dissociated eye movement, increased tendon reflexes of the extremities, Babinski reflexes, inconti-nence of urine and generalized seizures followed. She finally fell into akinetic mutism and died of acute bronchopneumonia. The total clinical course was thirteen years.

The brain weighed 880gm. Macroscopically, the brain was atrophic in general appearance. On the coronal sections, the cerebral cortex was reduced in thickness. The cerebral white matter was a-trophic with dilatation of the entire ventricular system. Histopathologically, a diffuse and sym-metrical demyelination, fiber gliosis, and damaged axis cylinders were observed in the cerebral white matter except for the U-fibers. There was no perivascular inflammation and metachromatic sub-stances. Large amounts of sudanophilic lipids and some gitter cells were scattered throughout the tissue without being concentrated around the blood vessels. Some of the glial cells contained lipopig-ments which showed a PAS positive and a positive iron reaction. Lots of senile plaques were scattered throughout the cerebral cortex but there was no Alzheimer's tangles in the nerve cells. Pyramidal tracts in the brain stem showed demyelination, gliosis, degeneration of the axons, and sudanophilic lipids. In other organs, fatty degeneration occurred in the liver.

Lipid analysis of the frontal white matter re-vealed that there was a marked decrease of glycol-ipid (cerebroside and sulfatide) and phosphatidyl ethnolamine.

This report reviews cases of sudanophilic leuco-dystrophy and the differences of the clinicopatho-logical findings between adrenoleucodystrophy and membranous lipodystrophy.