[Clinocopathological conference. IX. Muscular weakness and skin edema].
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A 75-year-old man was admitted because of chest pain and gait disturbance. He noticed pain in the left anterior chest regardless of exercise 3 months prior to admission. Two months later, the chest became smart with motion, followed by sensory disturbance of the lower extremities. On admission, he was anemic. His lower extremities showed exaggerated deep tendon reflexes with positive Babinski signs and an impairment of superficial and deep sense with sensory ataxia. Muscular weakness, muscular wasting, or urinary disturbance was not found. An X-ray film of his thoracic spine disclosed compression fracture and protein content of his cerebrospinal fluid was increased. Magnetic resonance image (MRI) of the ill spines revealed fusion of the bodies of the fifth and sixth thoracic vertebrae with a low signal intensity on T1-weighted images and a partially high signal intensity on T2-weighted images. The intervertebral disc was destroyed. Although histological or bacteriological confirmation was absent, characteristic MRI findings were compatible with spinal osteomyelitis. After antibacterial chemotherapy, his clinical symptoms improved and he came to be able to walk again.
Eleven horses (3 mares, 7 stallions, 1 gelding) with clinical and biochemical evidence of hyperkalemic periodic paralysis were studied. Each horse had history of episodic weakness, muscular tremors, or collapse, which lasted for periods of a few minutes to hours. Diagnosis was based on hyperkalemia in association with a spontaneous episode of paralysis or by precipitation of an episode by oral administration of potassium chloride. Clinical and biochemical events were documented during spontaneous and induced episodes of muscular weakness. During episodes, electrocardiographic findings were consistent with hyperkalemia. Electromyography performed between episodes revealed fibrillation potentials and positive sharp waves, complex repetitive discharges, and myotonic discharges. Histologic changes in muscle biopsy specimens varied from no overt changes in some horses to vacuolation in type-2B fibers with mild degenerative changes in other horses. Electron microscopy of myofibers revealed dilatations of the sarcoplasmic reticulum. Analysis of blood samples taken serially during induced attacks in 5 horses revealed marked hyperkalemia (5.5 to 9.0 mEq/L), with normal acid-base status, hemoconcentration, and modest changes in muscle-derived enzymes. Close correlation (r2 = 0.882) between total plasma protein and plasma potassium concentrations was observed and indicated a shift of fluid out of the extracellular fluid compartment. Treatment of either spontaneous or induced episodes by IV administration of calcium, glucose, or bicarbonate resulted in rapid recovery. Dietary management or daily administration of acetazolamide effectively controlled episodes. An affected mare was bred to an affected stallion, and 3 affected offspring were produced by embryo transfer. Blood samples from another extended family of affected horses were analyzed for identification of a genetic marker.(ABSTRACT TRUNCATED AT 250 WORDS)
The patient is a 73-year-old male. In January 1988, he noticed sensory disturbance and muscular weakness of his extremities. His thymoma was found in July 1988, and in October 1988, he was admitted to our hospital for treatment. Neurological examination showed muscular weakness, muscular atrophy, fasciculation, and distal dominant sensory disturbance of his right upper extremity and his bilateral lower extremities. The immunological examination showed an increase in serum IgG, gamma-globulin, OKT3 and OKT4 lymphocytes. Electromyographic studies showed severe waxing by repeated stimulations (200% by 10 Hz; 330% by 20 Hz), very low M wave amplitude by single stimulation, and post-tetanic exhaustion. An Eaton-Lambert-type neuromuscular transmission block was thus thought to exist. Concentric needle electromyography showed a neurogenic pattern. The sensory and motor nerve conduction velocity was delayed. Microscopic examination of biopsied skeletal muscle showed neurogenic change. A sural nerve biopsy specimen showed a decrease in the number of myelinated fibers (fiber density 4333/mm2), segmental demyelination, remyelination, and mild axonal change. Thymectomy was performed and pathological examination showed invasive thymoma of predominantly the epithelial type. His muscular weakness and sensory disturbance improved gradually after thymectomy. On the postoperative immunological examination, serum IgG, gamma-globulin, OKT3 and OKT4 lymphocytes were normalized. On the other hand, OKT8 lymphocytes were increased and the OKT4 to OKT8 ratio was decreased. The electromyographic studies showed no waxing, normal M wave amplitude, and no post-tetanic exhaustion. Therefore, we consider that an Eaton-Lambert-type neuromuscular transmission block and sensorimotor neuropathy in this case were caused by the immunological abnormality accompanying malignant thymoma.
BACKGROUND: The incidence, etiology, and evolution of complications after interscalene brachial plexus block (ISB) are not well-known. The authors prospectively monitored 521 patients for complications during the first 9 months after ISB. METHODS: A total of 521 adults scheduled for elective shoulder surgery performed with an ISB were included in this prospective study. The ISB procedure was standardized for all patients Acute complications were recorded. Patients were observed daily (for 10 days) for paresthesias, dysesthesias, pain not related to surgery, and muscular weakness and were evaluated at 1, 3, 6, and 9 months after surgery. Persistence of paresthesias dysesthesias, pain not related to surgery, or muscular weakness was investigated at 1 or 3 months by means of electroneuromyography. Final evaluation was performed at 9 months. RESULTS: A total of 520 patients completed the study; one was excluded after surgical axillary nerve damage. Two hundred thirty-four patients had an interscalene catheter. Acute complications consisted of one pneumothorax (0.2%) and one episode of central nervous system toxicity (incoherent speech; 0.2%). A 10 days, 74 patients (14%) were symptomatic, and none had muscular weakness. At 1 month, 41 patients (7.9%) had symptoms, and none had muscular weakness. Thirty patients under went electroneuromyography; sulcus ulnaris syndrome (n = 8) carpal tunnel syndrome (n = 2), and complex regional pain syndrome (n = 1) were diagnosed. At 3 months 20 patient (3.9%) were symptomatic, and none had muscular weakness All underwent electroneuromyography; carpal tunnel syndrome (n = 2), complex regional pain syndrome (n = 4), plexus neuropathy (n = 1), and plexus damage (n = 1) were diagnosed. At 6 months, 5 patients (0.9%) were symptomatic. At 9 months 1 patient (0.2%) had persistence of dysesthesia. CONCLUSIONS: Interscalene brachial plexus block performed with a standardized technical approach, material, and drugs is associated with an incidence of short- and severe long-term complications of 0.4%. In case of persistent paresthesia, dysesthesia, or pain not related to surgery after ISB, sulcus ulnaris syndrome, carpal tunnel syndrome, or complex regional pain syndrome should be excluded since specific treatment may be required.
We report the clinical and bioptic findings for a 57-year-old woman with severe chloroquine-induced myopathy. Since 1989, she had been suffering from systemic lupus erythematosus (SLE) with renal involvement and undergone periods of treatment with azathioprine and cyclophosphamide. Additional therapy with chloroquine (CQ) was started because of arthralgia. At the same time, slightly increased creatine kinase (CK) levels were noted. Myositis was suspected, and the patient was treated with steroids. The CK increase persisted, however, and she developed progressive muscular weakness and muscular atrophy. Routine controls revealed markedly elevated CK levels of 1,700 U/l. The neurological and electrophysiological findings were not typical of myositis. Thus, muscle biopsy of the deltoid muscle was performed in order to exclude polymyositis or toxic myopathy. As it revealed chloroquine-induced myopathy, medication was stopped. Discriminating between primary SLE-induced affection of the musculoskeletal system and drug-induced side effects is important for appropriate treatment of SLE patients.
We report a 14-year-old girl with early onset of slowly progressive muscular weakness and atrophy. There was no family history of neuromuscular disease. A persistent increase of serum creatine kinase was found. Muscle biopsy specimens showed type 1 fiber predominance and tubular aggregates in almost every fiber. The clinical findings and pathology suggest that the disease represents one variant in a group of rare myopathies with different patterns of inheritance, characterized by slowly progressive muscle weakness and tubular aggregates.
This study investigated and compared the gait of two patients with spinal muscular atrophy, type II (SMA II) and two patients with Duchenne muscular dystrophy (DMD). These diseases cause a progressive and proximal to distal muscular weakness resulting in the loss of ambulation. The DMD cases had comparable muscle weakness with the SMA II cases on manual muscle testing and patients were assessed using kinematics, kinetics, electromyography and video analysis. SMA II and DMD patients employed different gait strategies for forward movement. SMA II patients used pelvic rotation initiated by the upper body to propel the leg forward and produce the necessary step-length whereas the DMD patients tended to use hip flexion and plantar flexion. Management of SMA II patients would include preservation of hip abductor and flexor strength to maintain mobility.
An autopsy case of myotonic dystrophy (MD) is reported. The patient was a 58-year-old male. He presented with muscular weakness and muscular atrophy at the age of 33 and was diagnosed as having MD from myotonic symptoms (i.e. percussion and grip myotonia) at 49 years old. Mental disorders including a delusional hallucinatory state, mental slowness, indifference, and lack of spontaneity as well as visual cognitive impairments were noted at the age of 55. He showed Parkinsonism and died of septic shock. T2-weighted magnetic resonance imaging demonstrated diffuse cortical atrophy with a marked frontal atrophy and high-intensity signals in the white matter. Single photon emission computed tomography demonstrated hypoperfusion in the frontal cortex. Neuropathologic observation revealed neuronal loss in the superficial layer of the frontal and parietal cortices and extensive neuronal loss in the occipital cortex, intracytoplasmic inclusion body in the nerve cell of the medial thalamic nuclei, neuronal loss and presence of Lewy bodies in the substantia nigra and locus ceruleus corresponding to the pathologic features of Parkinson's disease, as well as abnormalities of myelin in the white matter. The present case suggests that in MD brain, various neuropathologic changes may occur and they contribute to the mental disorders.
Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motoneuron disease caused by a CAG-repeat expansion in the androgen receptor (AR) gene and for which no curative therapy exists. However, since recent research may provide opportunities for medical treatment, information concerning the natural history of SBMA would be beneficial in planning future clinical trials. We investigated the natural course of SBMA as assessed by nine activities of daily living (ADL) milestones in 223 Japanese SBMA patients (mean age at data collection = 55.2 years; range = 30-87 years) followed from 1 to 20 years. All the patients were diagnosed by genetic analysis. Hand tremor was an early event that was noticed at a median age of 33 years. Muscular weakness occurred predominantly in the lower limbs, and was noticed at a median age of 44 years, followed by the requirement of a handrail to ascend stairs at 49, dysarthria at 50, dysphagia at 54, use of a cane at 59 and a wheelchair at 61 years. Twenty-one of the patients developed pneumonia at a median age of 62 and 15 of them died at a median age of 65 years. The most common cause of death in these cases was pneumonia and respiratory failure. The ages at onset of each ADL milestone were strongly correlated with the length of CAG repeats in the AR gene. However CAG-repeat length did not correlate with the time intervals between each ADL milestone, suggesting that although the onset age of each ADL milestone depends on the CAG-repeat length in the AR gene, the rate of disease progression does not. The levels of serum testosterone, an important triggering factor for polyglutamine-mediated motoneuron degeneration, were maintained at relatively high levels even at advanced ages. These results provide beneficial information for future clinical therapeutic trials, although further detailed prospective studies are also needed.