[Neurologic and psychiatric syndromes of rare para-rheumatic diseases (author's transl)]. 1977

W Gottwald

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...

UI MeSH Term Description Entries
D009220 Myositis Inflammation of a muscle or muscle tissue. Inflammatory Myopathy,Myositis, Focal,Myositis, Infectious,Idiopathic Inflammatory Myopathies,Idiopathic Inflammatory Myopathy,Idiopathic Inflammatory Myositis,Infectious Myositis,Inflammatory Muscle Diseases,Inflammatory Myopathies, Idiopathic,Inflammatory Myopathy, Idiopathic,Muscle Diseases, Inflammatory,Myopathies, Idiopathic Inflammatory,Myopathy, Inflammatory,Myositis, Proliferative,Focal Myositides,Focal Myositis,Infectious Myositides,Inflammatory Muscle Disease,Inflammatory Myopathies,Muscle Disease, Inflammatory,Myopathies, Inflammatory,Myopathy, Idiopathic Inflammatory,Myositides,Myositides, Focal,Myositides, Infectious,Myositides, Proliferative,Proliferative Myositides,Proliferative Myositis
D009422 Nervous System Diseases Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle. Neurologic Disorders,Nervous System Disorders,Neurological Disorders,Disease, Nervous System,Diseases, Nervous System,Disorder, Nervous System,Disorder, Neurologic,Disorder, Neurological,Disorders, Nervous System,Disorders, Neurologic,Disorders, Neurological,Nervous System Disease,Nervous System Disorder,Neurologic Disorder,Neurological Disorder
D001933 Brain Stem The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA. Brainstem,Truncus Cerebri,Brain Stems,Brainstems,Cerebri, Truncus,Cerebrus, Truncus,Truncus Cerebrus
D003095 Collagen Diseases Historically, a heterogeneous group of acute and chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis, etc. This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (From Cecil Textbook of Medicine, 19th ed, p1494) Collagen Disease,Disease, Collagen,Diseases, Collagen
D006103 Granuloma, Lethal Midline A condition that is characterized by inflammation, ulceration, and perforation of the nose and the PALATE with progressive destruction of midline facial structures. This syndrome can be manifested in several diseases including the nasal type of EXTRANODAL NK-T-CELL LYMPHOMA and GRANULOMATOSIS WITH POLYANGIITIS. Granuloma Gangraenescens,Lethal Midline Granuloma,Polymorphic Reticulosis,Granulomas, Lethal Midline,Lethal Midline Granulomas,Midline Granuloma, Lethal,Midline Granulomas, Lethal,Polymorphic Reticuloses,Reticuloses, Polymorphic,Reticulosis, Polymorphic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001528 Behcet Syndrome Rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon. The neuro-ocular form may cause blindness and death. SYNOVITIS; THROMBOPHLEBITIS; gastrointestinal ulcerations; RETINAL VASCULITIS; and OPTIC ATROPHY may occur as well. Behcet Disease,Behcet's Disease,Behcet's Syndrome,Triple-Symptom Complex,Adamantiades-Behcet Disease,Behcet Triple Symptom Complex,Behçet Disease,Old Silk Route Disease,Triple Symptom Complex,Adamantiades Behcet Disease,Adamantiades-Behcet Diseases,Behçet Diseases,Symptom Complex, Triple,Triple Symptom Complices
D012859 Sjogren's Syndrome Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis. Sicca Syndrome,Sjogren Syndrome,Sjogrens Syndrome,Syndrome, Sicca,Syndrome, Sjogren's
D014890 Granulomatosis with Polyangiitis A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and KIDNEYS. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against MYELOBLASTIN. Granulomatosis, Wegener's,Wegener Granulomatosis,Wegener's Granulomatosis,Granulomatosis with Polyangiitides,Granulomatosis, Wegener,Polyangiitides, Granulomatosis with,Polyangiitis, Granulomatosis with,with Polyangiitides, Granulomatosis,with Polyangiitis, Granulomatosis
D019965 Neurocognitive Disorders Diagnoses of DEMENTIA and AMNESTIC DISORDER are subsumed here. (DSM-5) Clerambault Syndrome,Delirium, Dementia, Amnestic, Cognitive Disorders,Kandinsky Syndrome,Mental Disorders, Organic,Organic Brain Syndrome, Nonpsychotic,Organic Mental Disorders,Organic Mental Disorders, Psychotic,Psychoses, Traumatic,Mild Neurocognitive Disorder,Nonpsychotic Organic Brain Syndrome,Disorder, Mild Neurocognitive,Disorder, Neurocognitive,Disorders, Mild Neurocognitive,Disorders, Neurocognitive,Disorders, Organic Mental,Mental Disorder, Organic,Mild Neurocognitive Disorders,Neurocognitive Disorder,Neurocognitive Disorder, Mild,Neurocognitive Disorders, Mild,Organic Mental Disorder,Traumatic Psychoses

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