[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.

Connection of microtubules, caveolae, mitochondria and sarcoplasmic reticulum in the taenia coli of guinea-pigs.

Microtubules were identified in the subsarcolemmal area of smooth muscle cell in the taenia coli of guinea-pigs. They may traverse myofibrils but more frequently they run parallel with the long axis of the cell. The microtubules were often in contact with sarcolemmal caveolae. A caveola appeared to contact with an end of a microtubule or with the outer membrane of a mitochondrion. Occasionally a long mitochondrion was located between two evaginations of sarcolemma. The direct contact of caveolae with a mitochondrion was typical in such long mitochondria. Granular and agranular sarcoplasmic reticulum (SR) were also in the vicinity of mitochondria. The SR was in contact with mitochondria and also with vesicles. Subsarcolemmal mitochondria were often in continuity with agranular SR or invaginations of sarcolemma. Low evaginations of sarcolemma appeared to be a cell contact device but nexas-like interdigitations, protrusions from the dark cell into the light cell, were also evident. These morphological findings may suggest a potential network of transport or conductance in this type of smooth muscle.

Intramitochondrial dense bodies in the goblet cells of the ileum of muscular dystrophic mice.

Mitochondrial osmiophilic bodies were found in the goblet cells of the small intestine in a group of muscular dystrophic mice. They were not found in any littermate normal control mice. The dense bodies were uniformly round in shape but variable in size, approximately 0.2-0.4 mu in diameter. A limiting membrane encircled a dense content. Mitochondria with such dense bodies were distributed throughout the goblet cells. Adjacent absorbing epithelial cells had none of such mitochondrial bodies. Though the site of formation of the mitochondrial bodies remains to be investigated, the Golgi complex did not appear to be involved in the transportation of the dense bodies. Digestion of sections with ether-chlorofolm (1:1) slightly decreased the density of the mitochondrial body. Ruthenium red staining was not visible over the dense bodies but trace of Ru was detected by X-ray microanalysis. X-ray microanalysis indicated very few, if any, amounts of P and Ca, and it was not probable that phospholipid and calcium binding protein were the main constitution of these dense bodies.