[Juvenile Binswanger-type encephalopathy with alopecia and spondylosis deformans--a case report]

The patient is a 33-year-old male and his parents are first cousins. He noticed his hair loss since about the age 14. At age 29, he manifested gait disturbance and urinary incontinence, which gradually progressed. Neurologically, he showed dementia (WAIS < 64), pyramidal and extrapyramidal signs, and pseudobulbar palsy. His blood pressure was normal. He also had dry skin with sclerema and marked cervical and lumbar spondylosis. His brain CT showed enlargement of the lateral ventricles and the periventricular low density areas. T2 weighted image of MRI showed diffuse high intensity in the periventricular white matter. Yamada et al presented a case progressive subcortical vascular encephalopathy (Binswanger type) with alopecia and spondylosis as a possible new syndrome, and this patient has the same syndrome. The etiology of this syndrome has not been known at the present time. The biopsied skin from the patient showed much hyaline deposits like glycoprotein in perivascular area of dermis. These morphological changes are very similar to those of lipoid proteinosis. These findings suggested that the pathological mechanism of this syndrome might be related to the biochemical disturbance in lipoid proteinosis.