Guillain-Barré syndrome in association with focal segmental glomerulosclerosis.
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Biomedical subjects
Publications and source records attributed to A Druschky.
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HISTORY AND CLINICAL FINDINGS: A 56-year-old woman with hepatitis C had symptoms of a polyneuropathy with asymmetrical distal pareses and painful sensory disturbances in the limbs. INVESTIGATION: In addition to positive serology for hepatitis C cryoglobulins were demonstrated and complement C4 was reduced. Biopsy of the sural nerve showed a vasculitic neuropathy. TREATMENT AND COURSE: The multiple neuropathy (cryoglobulinaemia neuropathy), associated with hepatitis C, was treated with corticosteroids (starting with 60 mg/d prednisolone) and the neurological abnormalities regressed well. CONCLUSION: Vasculitis associated with hepatitis C should be included in the differential diagnosis of peripheral neuropathy.
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A 45 year old woman is reported who initially presented with a cerebellar syndrome, severe ataxia, and dysarthria. She rapidly deteriorated to coma vigile with bilateral myoclonic jerks, flexion rigidity, and immobility necessitating complete nursing. Her EEG showed generalised slow activity and periodic biphasic and triphasic waves. The CSF concentration of neuron specific enolase was very high. Consequently the diagnosis of Creutzfeldt-Jakob disease was established. Eight months later she died of respiratory complications. Thirty years earlier the patient had undergone corneal transplantation for keratoconus. Review of the organ donor's hospital records showed that death was caused by intercurrent pneumonia subsequent to subacute spongiform encephalopathy confirmed by necropsy. In view of two previous case reports in the literature it is presumed that the cadaveric cornea was the source of transmission of Creutzfeldt-Jakob disease in this patient.
BACKGROUND: Diagnosis of bacterial meningitis is due to granulocytic pleocytosis of CSF. In developing countries and in hospitals without continuous availability of a clinical laboratory a bedside-method of diagnosing meningitis is desired. METHODS: 75 CSF samples were tested for granulocytes and protein with reagent strips and the results were compared with those obtained from the clinical laboratory. RESULTS: All 48 samples with normal cell count were correctly diagnosed negative using reagent strips. Twenty-five samples of 27 samples with granulocytic pleocytosis were correctly recognised as right positive (sensitivity 92.6%; specificity 100%). All 48 samples with normal protein were correctly tested normal. Twenty-one samples of 27 samples with elevated protein were correctly diagnosed right positive (sensitivity 77.8%; specificity 100%). CONCLUSION: Testing CSF for granulocytes and protein with urine reagent strips is a bedside-method which allows a rapid and reliable decision whether CSF is normal or granulocytically pleocytotic.
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Crohn's disease is a chronic relapsing inflammatory disorder primarily affecting the gastrointestinal system. Extraintestinal manifestations such as erythema nodosum, iridocyclitis, arthritis and sclerosing cholangitis are frequent. We report on a 57-year old male patient with the symptoms of Crohn's disease. Clinically he showed signs of a muscle disorder and muscle biopsy demonstrated a perimysial inflammation with lymphocytes and histiocytes. Complete remission was achieved by immunosuppressive treatment. Six cases presented in the literature are reviewed and the autoimmunological aspects of the pathogenesis discussed.
Remote adverse effects of local intramuscular botulinum toxin were investigated in a prospective follow-up study. Twenty-six patients with spasmodic torticollis were examined (18 women, eight men, 45 +/- 13 years). Respiratory heart rate variation (HRV) was investigated by a computerized method. Different parameters were recorded (beats per minute, coefficient of variation, root mean square successive difference (RMSSD), spectral analysis, difference and quotient between maximum and minimum RR intervals, mean circular resultant). After one intramuscular injection of 12.5 ng botulinum toxin (Porton Products Ltd., England), no significant influence on HRV was seen. After the second injection, a significant attenuation was seen of four parameters (coefficient of variation, Rmax - Rmin, Rmax divided by Rmin, mean circular resultant) that lasted up to several months. No clinically manifest remote side effects and no cardiac arrhythmia were seen for several months of botulinum toxin treatment. Our investigation proves an effect of local intramuscular botulinum toxin on autonomic cardiac innervation.