[Botulinum toxin therapy in rehabilitation neurology]. / Botulinumtoxintherapie in der Rehabilitationsneurologie.

Intramuscular injections of botulinum toxin (BT) induce a localized, long-lasting paresis of the target muscle that can be graded by the BT dose administered and that is free of major side effects. With this profile, BT can be used for symptomatic treatment of various disorders caused by pathological muscle hyperactivities. These disorders occur frequently in patients in institutions for neurological rehabilitation. They are difficult to treat with conventional methods, can cause major suffering and induce substantial costs for our health care system. In the present study we therefore sought to delineate the use of BT in rehabilitative neurology. Regardless of the etiology of the particular muscle hyperactivity syndrome, five main indication groups are identified: (1) improvement of function of the hyperactive muscles; (2) relief of pain in the hyperactive muscles; (3) improvement of patient care; (4) avoidance of sequelae in joints, tendons, ligaments, and teeth; (5) various special indications.

[Differential diagnosis of idiopathic facial paralysis: Bannwarth meningopolyradiculitis]. / Zur Differentialdiagnose der idiopathischen Facialisparese: Meningopolyradikulitis Bannwarth.

Lymphocytic meningopolyradiculitis (Bannwarths' syndrome) is a tick-borne Borrelia infection of man. About 60 per cent of such patients exhibit a peripheral facial paresis whose characteristics are clinically not distinguishable from Bell's paresis. Of major importance for the diagnosis, besides the radicular pain that nearly always prevails, are particularly further motoric deficits, a previous erythema migrans and remembered thick bites. A tentative diagnosis can be verified by liquor examination which reveals a characteristic pattern of protein distribution and lymphocytic pleocytosis. Penicillin in high doses is the therapy of choice.

[Solitary neurofibroma of the lumbosacral plexus. Case report]. / Solitäres Neurofibrom des Beinplexus (Plexus lumbosacralis). Ein Fallbericht.

The article describes a case of a solitary neurofibroma of the plexus lumbosacralis causing pain and incomplete leg paresis. Complete relief was obtained by total extirpation of the tumour, a procedure which must be regarded as the method of choice. Pain and pareses in the lower extremities require differential diagnostic consideration of rare neurogenic tumours of the peripheral nervous system in the regions of the pelvis and plexus lumbosacralis. Neurological examination by a specialist is of significant importance preoperatively to determine the seat of the tumour and to assess the postoperative course.

Adult metachromatic leukodystrophy. IV. Ultrastructural studies on the central and peripheral nervous system.

Ultrastructural studies on the central and peripheral nervous system of 2 patients with adult onset metachromatic leukodystrophy (MLD), dead at the ages of 46 and 51 years, showed MLD-specific inclusions, tufaceous and prismatic structures, a wide spectrum of membranous arrangements within lysosomal residual bodies, and the intimate admixture of sulfatides and other membranous material with lipopigments. Oligodendrocytes and Schwann cells were foremost affected but membranous inclusions could also be verified in neuronal perikarya and astrocytes. The varying ultrastructural spectrum of lysosomal residual bodies in adult onset MLD and the association with lipopigments, chiefly in nerve cells, exceed the fine structural observations on late infantile and juvenile MLD and may reflect morphological differences between these subtypes of MLD that are also known from clinical and biochemical observations.

The ultrastructure of the retina in adult metachromatic leukodystrophy.

A 46-year-old woman afflicted with biochemically proven metachromatic leukodystrophy had only mild optic atrophy shortly before her death. Repeated earlier ophthalmoscopic examinations had not revealed any retinal abnormalities. Light microscopy of the retina showed strong acid phosphatase activity in both enlarged ganglionic cells and pigment epithelial cells. Demyelination of both optic nerves was not noted. Ultrastructurally, membranous lysosomal residual bodies were confined to ganglionic cells. We found lipofuscin material in pigment epithelial cells, but also within metachromatic leukodystrophy-specific residual bodies of ganglionic cells. The presence of lipofuscin represents the "wear-and-tear" phenomenon, possibly enhanced by the metachromatic leukodystrophy.

Ultrastructural findings of peripheral nerve in a preclinical case of adult metachromatic leukodystrophy.

In a 13-year-old neurologically healthy boy from a family with adult-onset of metachromatic leukodystrophy (MLD) showing arylsulfatase A-deficiency in the adult, sural nerve biopsy probably was performed 2-3 decades before clinical manifestation of the disease could be expected. Ultrastructurally 4 basic types of inclusion bodies in Schwann cells could be demonstrated (pleo-morphic "zebra body"-like inclusions, double-lamellated inclusions, "tuff-stone"-like inclusions, granular osmiophilic inclusions). Additionally, endoplasmatic reticulum, mitochondria and lysosomes showed marked alterations. Advanced damage of myelin was only rarely seen, but initial segmental demyelination was a common finding. These early pathological changes in chronic MLD are thought to represent a subcellular metabolic insufficiency of Schwann cells in this disease.

Adult metachromatic leukodystrophy. II. Ultrastructural findings in peripheral nerve and skeletal muscle.

Sural nerve biopsy in a 44-year-old woman with adult metachromatic leukodystrophy (MLD) confirmed by deficient arylsulfatase-A activity, showed a reduction in the number of large and small myelinated axons, and sparse metachromatic material. Ultrastructurally, the latter consisted of various types of residual bodies including the tufaceous and prismatic forms typical of MLD. In the striated muscle, large amounts of regular lipofuscin but no MLD-characteristic inclusions were encountered. Inclusion-bearing mitochondria in the muscle appeared to be an incidental finding.

[Radiculo-tomography for lumbosacral myelography (author's transl)]. / Radikulotomographie bei der lumbosakralen Myelographie

The use of tomography can increase the value of lumbosacral myelography in certain circumstances. Post mortem studies have resulted in standardisation of the examination, which will combine optimal diagnostic information with the least amount of effort and with an acceptable amount of stress on the patient. 110 tomographic examinations were performed amongst 680 lumbosacral myelograms (Dimer X). On the basis of this experience, the value of this procedure is illustrated from various points of view and its indications are discussed.

Early sterile autopsy in etiological studies on multiple sclerosis.

The clinical findings and course in the first 2 cases of multiple sclerosis are described, in whom it was possible to isolate a virus from brain tissue by early sterile autopsy and fusion technique. It is noteworthy that in one of these cases multiple sclerosis probably occurred in female members of the family through three consecutive generations. A report is made on an additional case, in which no virus could be isolated, but in which electron microscopic studies showed two types of virus particles: nucleocapsid-like structures and particles identical in form and size with papova virus. Electron microscopic findings and attempts to cultivate a virus in multiple sclerosis published by other authors are discussed. It is pointed out, that the results described provide as yet no proof for the viral etiology of multiple sclerosis. They support the hypothesis, however, that a virus may play an essential role in the etiology and/or pathogenesis of this disease. Problems in relation to brain autopsy in multiple sclerosis are discussed. Early sterile autopsy is considered the most practicable possibility for obtaining tissue material for culturing and for ultrastructural studies in multiple sclerosis.