Heidenhain病の1例について

A Case of Heidenhain's Syndrome

I．症例

［望○元　24才，男子，工員］

　家族歴：両親健在，同胞6名健康。

A 24 year old male, developed repeated hallu-cinatory-delusional state with psychomotor excite-ment and incidiously progressed dementia.

He developed suddenly neurological symptomrsincluding ataxia, dysarthria, dysphagia, hyperhy-drosis, fever, pyramidal and extrapyramidal associ-ated with mental deterioration before 2 months ofhis death.

From the very beginning of the above mentionedsymptomes, he became semicomatose and remainedin that state until he deceased after 4 years and 7months.

The autopsy revealed bilateral lamellar changeswhich consisted of status spongiosus in the calca-rine region of the occipital lobes. Histologically,diffuse cortical degeneration and status spongiosusparticular emphasis on the occipital cortex werefound.

The loss of nerve cells wore fleck-like appea-rance, and remainning cells showed the severe cellchange.

In the all lobes, marked astrocytic proliferationand hypertrophy, marked microglial profileration,and acute swelling of oligodendroglia were observed,especially in the occipital lobe.

To mention specifically, there were round "Luc-kenherd" with indistinct border in the precentralregion, in which only macroglia had remained.

In the globus pallidus, the small nerve cells weremainly affected with moderate macroglial prolifer-ation. In the cerebellum, there were slight loss ofPurkinje cells and moderate proliferation of Berg-mann's astrocytes.

The condition resembles so closely to what previ-ously was reported by Heidenhain and describedby Meyer as "Heidenhain's syndrome".

The auther considered this as a case of Heiden-hain's syndrome.