[Neurological, neurophysiological, neuropathological and psychiatric aspects of scleroderma].
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Biomedical subjects
Publications and source records attributed to W Gottwald.
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A general review on vasculitis nodosa of report in literature since the work of Strammler 1958 is given. The neurological and psychiatric manifestations of the disease are completely dealt with, whereas dermatological, ophthalmological and otological aspects are brieftly mentioned. Varieties of diffuse and localized cerebral, as well as peripheral syndromes are described. The neuropathological findings are also discussed extensively.
In Wegener's granulomatosis peripheral nerves are more often involved than central nervous structures; functional psychoses are secondary e.g. to renal complications. Neurologic-psychiatric symptoms are varied and result from local spread from the upper respiratory tract with separate granulomata and vasculitis. Striped muscle is involved in about 10% of cases. Specific vegetative disturbances of function do not appear to occur. This is also true for Granuloma gangrenescens. Its multicentric inflammatory, ulcerating and necrotic foci involve midline structures and often start at the anterior neuroporus. Becet's disease, however, shows clear characteristic localisation, symmetry of various dermatologic phenomena in the extremities and the almost obligatory involvement of mouth, stomach and the genitals and their surroundings. This one may assume to be due to a mesencephalic parasympathicotonic reaction as the basis for the occurrence of perioral and acro-syndroms after Fischer-Brügge and Sunder-plassmann. Some similarity with the localization of other collagenous diseases is evident. The anterior basal ganglia represent a favored focus where e.g. initial, imcomplete and readily recurrent processes, possibly of an exsudative nature, may start off neurovascular changes of limited areas without necessarily producing clinically detectable signs of the usual neurologic-psychiatric type. In Behcet's disease, rather as in scleroderma, the nervous system appears to be decisively involved. About one third of the cases show changes in the nervous system ("Neuro-Behcet" in the usual sense) with little peripheral signs and rare myositis. But Behcet's disease may also-like erythematodes or progressive sclerodermia-simulate a disseminated encephalomyelitis. The pathogenetic factors of Reiter's syndrome appear to be closely related. The relatively constant main symptoms are also localized and permit the conclusion that they depend on vasomotorically linked innervation of the first bulbar segment. The preference for distal localization of bilateral skin involvement hints - as in Behcet's disease at hypothalamic distribution; main localization in midface and the oral mucosa, in eyes and the ends of the midline and, often, also involvement of certain midline and, often, also involvement of certain favored joints permit the assumption of mutural relations between the cranial and sacral fields of incidence of the trophotrop-endophylactic system which is linked via the parasympathic with periphery. According to present knowledge the bundles of Schütz and, further, Krüke may be the pathways. Where pelvic processes predominate viscero-cutaneous or cuto-visceral reflexes may - as in dermatomyositis - be involved. E.g visceroreflectory changes explain coincidences of signs in similar or adjacent segments of the urogenital system, the rectum and certain joints. Psychose on a physical basis are rare. As in other collagen diseases the spinal cord is rarely involved, but "multiple sclerosis" may be simulated...
Lupus erythematodes is related to the vegetative nervous system in Raynaud's disease, the butterfly distribution in the face, the involvement of the lateral part of the eyebrows (Hertoghe) and in cerebral attacks. Involvement of peripheral and central animal nervous systems is common and produces primary and secondary syndromes. Prominent are polymyositis or lupus-myopathy, in the brain mainly functional psychoses, epileptiform attacks, and a variety of focal, often very massive, signs. There are very clear differences from the neurologic signs of dermatomyositis, less so from arteritis nodosa and even less from progressive sclerodermia. Peripheral neurologic syndromes in lupus erythematosus are less common and more subtle than in arteritis nodosa. Bio-electric parameters of the petitmal-trias have been found. The relative absence of collagenoses from the spinal cord is also noticed in lupus erythematosus. Signs of involvement here appear to be limited and often subacute. Differential diagnosis has to consider many neurologic diseases, a special problem when MS is simulated just as in sclerodermia. Since cerbral attacks appear early in about 5%, the use of anticonvulsive drugs, particularly of the hydantoin group, provides special problems. Signs of myasthenia demand further attention. The neuropathologic changes are known in essential points and can obviously hardly be mistaken for inflammatory processes due to other causes. Certain basic facts appear to apply to other collagenoses. All 4 "grand" or "classic" collagen diseases are very similar and have much in common, but also show more or less definite differences. This is true for their clinical and anatomical appearance and last but not least to their neurologic aspects. The "roof-concept" of Collagenosis" is once more proved to be justified. As far as is known today the autonomic system plays a decisive and obligatory role only is sclerodermia. This syndrome is also the only one which contains the odd phenomenon of atrophy of which the best-known form is facial hemia-atrophy.
Paroxysmal syndromes do not occur frequently in the course of multiple sclerosis, but require diagnostic considerations of particular nature. The pathogenesis and clinical aspects of a) cerebral convulsions, b) (usually appearing unilaterally) tonic brain stem seizures, c) narcoleptic attacks, d) hemiballismus, e) acute attacks of vertigo, f) paroxysmal dysarthria, g) trigeminal neuralgia are discussed.
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Undesirable and usually reversible side effects in the treatment of convulsive diseases with preparations containing hydantoin most frequently arise as cerebellar atactic syndromes and more seldom in extrapyramidal hyperkinesias, parkinsonisms, manifold "encephalitic" forms, hemiplegias and abnormalities in the EEG, among others. The differential diagnosis of the reversible psychopathological and the rare irreversible (defect) syndromes is often difficult. The problems of correlation between psychoses arising from epilepsy and electroencephalographic parameters are pointed out and it is emphasized that the relation between the frequency of a convincing therapeutic effect and the frequency of manifestation of threatening intolerances remains relatively favorable for the hydantoins.
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