ABSTRACT
Weakness after exercise is not a commonly recognized major symptom of paramyotonia congenita. A patient with classic paramyotonia congenita developed thyrotoxicosis and severe weakness after exercise. This symptom resolved after the thyrotoxicosis was treated.
Subject(s)
Fatigue/complications , Hyperthyroidism/complications , Myotonia Congenita/complications , Physical Exertion , Adult , Cold Temperature/adverse effects , Fatigue/etiology , Female , Humans , Myotonia Congenita/physiopathologyABSTRACT
We report a case of inclusion body myositis and chronic immune thrombocytopenia with serum platelet antibodies and circulating immune complexes. Although immune mechanisms probably play an important role in the pathogenesis of inclusion body myositis, a viral etiology cannot be excluded.
Subject(s)
Antigen-Antibody Complex/immunology , Inclusion Bodies/ultrastructure , Myositis/complications , Thrombocytopenia/complications , Antibodies/analysis , Blood Platelets/immunology , Chronic Disease , Humans , Male , Middle Aged , Muscles/pathology , Muscles/ultrastructure , Myositis/immunology , Myositis/pathology , Thrombocytopenia/immunologyABSTRACT
A 41-year-old man survived 23 years with quadriplegia following a C-5 fracture dislocation. During the last four months of his life he had paroxysmal episodes of unconsciousness. Necropsy disclosed a posttraumatic syrinx. The cyst extended caudally to the midlumbar region and rostrally into the midline of the medulla without communication into the fourth ventricle. Central apnea due to expansion of the cyst in the medullary respiratory centers is postulated as the possible cause of his paroxysmal episodes of unresponsiveness.
Subject(s)
Syringomyelia/pathology , Adolescent , Apnea/etiology , Brain Diseases/etiology , Humans , Male , Medulla Oblongata/pathology , Spinal Injuries/complications , Syringomyelia/complications , Unconsciousness/etiologyABSTRACT
Facial or limb myokymia was present in 8 patients (17%) of 48 consecutive cases of Guillain-Barré syndrome. It occurred early in the course of the illness and persisted 5 to 40 days as the patients recovered. Facial myokymia usually occurred in clinically weak muscles, was most often bilateral, and was more common in women. Bilaterality and transient nature differed from the facial myokymia seen in pontine tumors and demyelinating disease.
Subject(s)
Myoclonus/complications , Polyradiculoneuropathy/complications , Adolescent , Adult , Aged , Extremities/physiopathology , Facial Muscles/physiopathology , Female , Humans , Male , Middle Aged , Myoclonus/physiopathology , Neural Conduction , Polyradiculoneuropathy/physiopathologyABSTRACT
This report outlines the clinical, electrophysiologic, and morphologic findings in seven patients with adult acid maltase deficiency. The diagnosis was confirmed biochemically in four of these individuals. Adult acid maltase deficiency must be considered whenever clinical diagnoses of polymyositis, especially the chronic form, and limb-girdle muscular dystrophy are being entertained.
Subject(s)
Glycogen Storage Disease Type II/pathology , Glycogen Storage Disease/pathology , Muscular Dystrophies/pathology , Myositis/pathology , Adult , Aged , Electromyography , Female , Glucan 1,4-alpha-Glucosidase , Glycogen Storage Disease/physiopathology , Humans , Male , Middle Aged , Muscular Dystrophies/enzymology , Myositis/enzymology , alpha-GlucosidasesABSTRACT
Maneuvers designed to manipulate ionized calcium (Ca++) were carried out in two patients with inflammatory polyradiculoneuropathy and myokymia. Increased clinical myokymia and myokymic burst amplification occurred when ionized Ca++ was lowered by plasma exchange or hyperventilation. Increasing ionized Ca++ (by intravenous infusion of CaCl2) decreased the myokymia. These findings indicate that myokymic discharges are altered by changes in serum ionized Ca++ and effects on axonal excitability.
