ABSTRACT
Myasthenia gravis is an autoimmune disease, which leads to load-dependent weakness of voluntary skeletal muscles with recovery of function after resting. The disease is caused by autoantibodies directed against the postsynaptic nicotinic acetylcholine receptors (AChR) leading to a reduction of neuromuscular transmission. Muscles and nerves are not affected. Disorders of the thymus play a role in the pathogenesis of AChR antibody-positive myasthenia. The clinical symptoms include exercise-induced fatigue either of the ocular muscles alone (ocular myasthenia) or striated skeletal muscle and the ocular, facial and bulbar musculature (generalized myasthenia). Treatment of myasthenia gravis involves administration of acetylcholine esterase inhibitors and immunosuppressive drugs. A myasthenic crisis is characterized by life-threatening complications with severe weakness, swallowing difficulties and respiratory failure, which requires intensive care treatment.
Subject(s)
Angiotensin-Converting Enzyme Inhibitors/administration & dosage , Diagnostic Techniques, Neurological , Immunosuppressive Agents/administration & dosage , Myasthenia Gravis/diagnosis , Myasthenia Gravis/therapy , Diagnosis, Differential , Evidence-Based Medicine , Humans , Treatment OutcomeABSTRACT
OBJECTIVES: During an epidemiological study of Creutzfeldt-Jakob disease in Germany, Hashimoto's encephalitis was encountered as a differential diagnosis, which has not yet been described in this context. METHODS: The symptoms and findings of seven patients who fulfilled the criteria for "possible" Creutzfeldt-Jakob disease are presented. RESULTS: A Hashimoto's thyroiditis with antibodies against thyroglobulin or thyroid peroxidase, or both and a hypoechoic thyroid ultrasonogram were found in all cases. Analysis of CSF disclosed an increased leucocyte count in three patients, and a raised CSF:serum concentration ratio of albumin (QA1b) in four patients. The 14-3-3 protein, typical of Creutzfeldt-Jakob disease, could not be detected in any of our patients. No periodic sharp wave complexes, which are typical of Creutzfeldt-Jakob disease, were detected on EEG in any of the cases. By contrast with Creutzfeldt-Jakob disease, which leads to death within a few months, the patients with Hashimoto's encephalitis often recover quickly when treated adequately. All the patients improved after administration of corticosteroids. CONCLUSION: The clinical symptomatology of both diseases may be very similar: dementia, myoclonus, ataxia, and personality change or psychotic phenomena are characteristic symptoms.
Subject(s)
Creutzfeldt-Jakob Syndrome/diagnosis , Thyroiditis, Autoimmune/diagnosis , Adult , Aged , Aged, 80 and over , Diagnosis, Differential , Female , Humans , Male , Middle AgedABSTRACT
Wernicke's encephalopathy (WE) is a consequence of vitamin B1 (thiamine) deficiency and in the majority of cases due to alcoholism. We report here the case of a 26-year-old male alcoholic who had stayed helplessly at home for 4 days until hospital admission. Clinical diagnosis was difficult due to major disturbance of consciousness. MRI showed an increase in signal intensity (T2-, FLAIR-weighted) around the third ventricle, the quadrigeminal bodies, the fornices, the mamillary bodies, the floor of the fourth ventricle and around the aqueduct. These findings were indicative of WE although of unusual extent. In this case MRI correlated well with clinical symptomatology. Therapy with thiamine was started immediately and symptoms as well as MRI findings resolved partially. The presented case illustrates the diagnostic usefulness of MRI in WE especially if the patient is of reduced consciousness and clinical investigation is limited.