Extreme, unilateral widening of Virchow-Robin spaces: case report.

We report a case of extreme, largely unilateral dilatation of Virchow-Robin spaces. Fluid-attenuated inversion-recovery images revealed high-signal foci adjacent to the dilated spaces, possibly due to chronic ischaemia.

Histogenesis of olfactory neuroblastoma. I. Electron microscopy of typical human case.

"Olfactory neuroblastoma" covers several types of esthesioneurogenic tumors such as esthesioneuroepithelioma, esthesioneuroblastoma and others. They are thought to be of olfactory mucosal origin and present with typical light microscopical "neurogenic features" e.g. rosettes and/or axon production. Some cases have been analyzed ultrastructurally and contained membrane bound granula like others tumors of the APUD system, originating from the neural crest. Furthermore neuronal differentiation of various degrees has been described. The human case of this contribution did not contain rosettes, but axons could be demonstrated in considerable number by silver impregnation. Electron microscopy could demonstrate the presence of dendritic processes with microtubuli and filaments as well as abundance of secretory granules of 1800 A. The comparison with the up to now published findings shows that the production of dense core granules is the most constant ultrastructural feature in these tumors; however, additional ultrastructural features in typical human cases as here presented, earlier experimental results and few human descriptions show, that DCV production is not essential for olfactory neuroblastoma. This might shed new light on the histogenesis of these tumors.

Alexander's disease in an adult: clinicopathologic study of a case and review of the literature.

A case of Alexander's disease in a 39-year-old man is reported. The clinical course was characterized by headaches and cerebellar gait for 6 months. A state of acute unconsciousness followed, lasting 2 days. Subsequently, dysfunction of the lower cranial nerves on the left-side, left-sided motor incoordination, and a right-sided spastic hemiplegia were found. The patient died 49 days after an acute incident of lung embolism. Neuropathologically generalized and focally pronounced Rosenthal fibers in subpial, subependymal, and perivascular regions were found. They were especially predominant in the brain stem and cerebellum. Atypical fibrillary glia, moderate patchy demyelination, and circumscribed cystic necrosis in the brain stem were also observed.

Infantile cortical measles inclusion body encephalitis during combined treatment of acute lymphoblastic leukemia.

A case of cortical measles inclusion body encephalitis occuring in a boy aged 6 years 7 months, 4 months after uncomplicated measles is reported. The child was undergoing combined treatment ofr acute lymphoblastic leukemia. He was in primary remission for 2 years. The neuropathological findings are characterized by necrosis, eosinophilic nuclear and cytoplasmic inclusion bodies in the neuronal and glial cells within the cerebral cortex and basal ganglia. Nucleocapsides of paramyxoviruses were detected in the nuclear inclusion bodies of both cell types.

The symptomatology, morphology and biochemistry of glycogenosis type II (Pompe) in the adult.

The mild, generalized myopathy (glycogenosis type II) of a 23-year-old male, previously thought to have progressive muscular dystrophy, was studied clinically, electro-myographically, biochemically and with light- and electron microscopes. However, the history and clinical aspects, as well as the registration of high frequency discharges in the electromyogram first made the diagnosis uncertain. This kind of spontaneous activity has been found in nearly all cases reported in the literature. Light microscopic and histochemical examinations show vacular degeneration and glycogen storage in muscle fibres. With the electron microscope we found free dispersed glycogen in the cytoplasm and membrane-bound glycogen, glycogen-filled lysosomes. Biochemical measurements of the muscle enzymes, involved in the glycogen breakdown, were normal except for acid alpha-1,4-glucosidase, which was deficient. The evidence of these findings in this abortive form of glycogenosis type II is discussed and compared with the few cases found in the literature.

The differential diagnosis of scapuloperoneal amyotrophy.

This report deals with a scapuloperoneal syndrome which developed simultanously with pain and distal paresthesias. In addition there was a slight sensory disturbance of glove and stocking type distribution. Motor conduction velocity was within normal limits and all distal latencies of response were normal; only the sensory conduction velocity of the left median nerve was found to be decreased (42.1 m/s). Electromyographic investigations revealed only signs of myopathy. Histological findings (m. deltoideus, m. tibialis anterior) favoured a primary myopathic process. Biopsy of the n. suralis revealed no certain pathological changes. The affection appears to have an autosomal dominant mode of inheritance. The sensory disturbance and decreased reflexes indicate an involvement of the nervous system, but the question of relationship to the scapuloperoneal muscular atrophy cannot yet be answered.

Quantitative histometric studies and relation of number and diameter of myelinated fibres to electrophysiological parameters in normal sensory nerves of man.

Quantitative histometric investigations were made in 6 radial and in 12 sural nerves of subjects without signs of a neuromuscular disorder. Nerve conduction velocities and amplitudes were studied on the same nerves. A linear relationship between the nerve fibre diameter and the conduction velocity of the fastest fibres was found. The conversion factor had a mean of 4.67, S.D. 0.16 in the radial nerves and an average value of 4.55, S.D. 0.19 in the sural nerves. Correlating the amplitudes of the compound nerve action potentials and the fibre number there was a linear proportionality between the log10 of number of fibres with a diameter of 11 mum and more, and the amplitude.

[Mitochondrial changes of the skeletal muscle in the peroneal muscular atrophy (Charcot-Marie-Tooth disease). Histological and electron microscopic studies (author's transl)]. / Mitochondrienveränderungen im Skeletmuskel bei neuraler Muskelatrophie (Charcot-Marie-Tooth). Vergleichende licht- und elektronenmikroskopische Untersuchung

This report deals with two sisters (38 and 44 years old) suffering from Charcot-Marie-Tooth disease. Muscle biopsies were taken from the deltoid and the rectus femoris. In one of the cases a sural nerve biopsy was also made. Light microscopy showed only slight myopathic changes. The histochemical reactions disclosed an increase in lipid deposition and in NADH-TR activity of type 1 fibres. Electron microscopy showed abnormal mitochondrial aggregates, which were surrounded inconstantly by glycogen deposits, especially in subsarcolemmal space. Many of the atypical mitochondria showed paracristalline inclusions within the cristae. The significance of these findings is discussed and compared with similar reports in the literature.