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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.

The purpose of the teleradiology project within the Marburg Image Management and Communication System (MIMACS) was to evaluate the teleconsultation process between the Departments of Diagnostic Radiology and Neuroradiology at the University Hospital of Marburg. The project was divided into three phases. In the first phase, completed in June 1992, the technical and organisational framework was developed. In the second phase, completed in October 1992, radiologists and neuroradiologists evaluated the functions of Siemens workstations DRC40 and Litebox by means of a questionnaire. It turned out that workstation functions most often used at both sites were: magnifying and rearranging images, displaying image data, using window/level presets and modifying the grayscale. Users rarely took advantage of the magic glass, zoom and density measurement functions. In the third phase of the study, the impact of teleconsultation on diagnoses and clinical recommendations was analysed. The study design included three radiologists who specified their diagnoses and clinical recommendations before and after consulting with the neuroradiologists. The degree of difference between these two points of judgement was evaluated by three radiologists who were not involved in the consultation process. The results of phase three show that teleconsultation changed the initial diagnostic findings in 34% of the cases and changed the clinical recommendation in 22% of the cases.

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 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.

"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.

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.

This report deals with the clinical and neuropathological findings in a case of Creutzfeldt-Jakob disease (subacute spongioform encephalopathy). A 60-year-old woman died after a 4 month illness characterized by a progressive dementia with pyramidal and extrapyramidal signs, myoclonic jerks and focal seizures in the left arm.

Klinik und neuropathologische Befunde bei metaneoplastischer Encephalitis werden an Hand zweier eigener Beobachtungen beschrieben. Einmal handelt es sich um einen 54jahrigen Mann, der an einem Bronchialcarcinom erkrankt war, zum anderen um eine 59jahrige Frau mit einem malignen Ovarialtumor. Bei beiden Patienten standen wahrend der 11- bzw. 18monatigen Krankheitsdauer cerebellare Symptome im Vordergrund. Histologisch fand sich das Bild eines ausgedehnten, perivasalen lymphocytar-entzundlichen Prozesses mit diffusen und knotchenartigen Gliareaktionen unter Bevorzugung des temporo-insularen Cortex, der Stammganglien des Hirnstammes und des Kleinhirns mit nahezu vollstandigem Untergang der Purkinjezellen.

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.

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.

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.

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.