Rhabdomyolysis triggered by an asthmatic attack in a patient with McArdle disease.

We describe a patient with McArdle disease who developed rhabdomyolysis triggered by a bronchial asthmatic attack. A 64-year-old man had chronic pulmonary emphysema with asthma, and an asthmatic attack led to severe rhabdomyolysis that required continuous hemodiafiltration. After 2 years, a physical examination revealed atrophy of the extremities compared with previous examinations, especially of the intercostal muscles. During that time, he suffered two severe bronchial asthmatic attacks. His serum level of creatinine kinase remained between 4,000 and 7,000 IU/l when he did not suffer from asthmatic attacks and rhabdomyolysis had abated. Therefore, we suspected that his recent muscle atrophy was caused by asthmatic attacks, and discussed the possibility of his respiratory muscle weakness due to McArdle disease in relation to his severe bronchial asthmatic attacks as well as chronic obstructive pulmonary disease.

Massive pulmonary hemorrhage due to cytomegalovirus infection in a Japanese patient with alpha-1-antitrypsin-deficient emphysema.

Although alpha(1)-antitrypsin (AAT) deficiency is one of the most common hereditary diseases and a recognized cause of emphysema in Caucasians, variants of this deficiency are extremely rare among Orientals. We present here a Japanese emphysema patient with the AAT deficiency variant originally identified as S(iiyama). After an 8-year follow-up period, the patient suffered from repeated pulmonary Pseudomonas aeruginosa infection for 4 years. He died suddenly of massive pulmonary hemorrhage. The pathologic examination revealed a necrotic hematoma in the right S10 lobe, which exhibited pneumonia due to cytomegalovirus (CMV) infection. Pulmonary hemorrhage due to CMV can occur and be fatal in patients with emphysema and AAT deficiency.

Pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia.

We describe the case of a 53-year-old Philadelphia-chromosome-positive woman with chronic myelogenous leukemia, who developed pulmonary alveolar proteinosis (PAP). The possible mechanism involved in the pathogenesis of PAP are discussed based on the clinical and laboratory data for this patient as well as on experimental and clinical data reported in the literature.

[Small cell lung cancer associated with subacute sensori-motor neuropathy in a patient whose symptoms subsided during chemotherapy].

A 71-year-old man was referred to us with diplopia, left peripheral facial nerve dysfunction, ataxic gait and dysesthesia of the extremities. Neurological examination revealed mild reduction of sensation to pinprick and light touch in the left dominant lower leg. His standing position was wide based, and he showed Romberg's sign. The patient also presented signs of left peripheral facial, bilateral abducent, and left oculomotor nerve dysfunction. Serum levels of CEA, CA 19-9, and proGRP were high. 67Gallium scintigraphy showed an accumulation of radioactivity at the hilum of the right lung, and the findings of bronchofiberscopy were compatible with the diagnosis of small cell lung cancer. Because the symptoms gradually worsened to the point that the patient could not move by himself, chemotherapy and radiotherapy were initiated 3 months after the onset of symptoms. While under chemotherapy, symptoms of neuropathy subsided and the patient was able to walk with the aid of a walking stick. Although all symptoms were indicative of carcinomatous neuropathy, no antineuronal antibodies were detected in the patient's serum by immunohistochemical techniques. However, because the lung cancer deteriorated gradually despite therapy, the patient died of respiratory failure. At autopsy, tumor metastases were found in the pericardium, left lung, both adrenal glands, right hilum lymph nodes, and mediasternal lymph nodes. No microscopic signs of metastases were found in the frontal, parietal, temporal, or occipital lobes, or in the basal ganglia, thalamus, midbrain, pons, cerebellar vermis and hemispheres, or upper medulla. Histopathologically, there was no degeneration of neuronal cell bodies in cerebellar or cervical dorsal root ganglia; however, almost total loss of myelinated fibers or variegated demyelination of myelinated fibers was observed in the anterior, lateral and posterior funiculus at both cervical segments of the spinal cord. The number of myelinated fibers was smaller in the 5th and 6th cervical left ventral roots. The reason why the patient's symptoms subsided during chemotherapy was probably a suppression of antineuronal antigen by chemotherapy and the repair of myelinated fibers.

Systemic lupus erythematosus with pulmonary hypertension.

A 50-year-old Japanese female with a long history of Raynaud's phenomenon presented with progressive dyspnea due to pulmonary hypertension. The diagnosis of systemic lupus erythematosus was confirmed by proteinuria, lymphocytopenia, bilateral pleurisy, and a seizure of convulsion which was consistent with neurological manifestations of systemic lupus erythematosus, whereas the antinuclear antibody showed a low titer. Despite improvement in the activity of systemic lupus erythematosus, steroid treatment did not alter the progression of pulmonary hypertension, which increased in severity, eventually resulting in her death. We believe pulmonary hypertension to be an unusual but critical complication of systemic lupus erythematosus.

Ganglioneuroblastoma with disseminated bone marrow infiltration in an adult.

Ganglioneuroblastoma is an extremely rare neoplasm of adults. We present a case of adrenal ganglioneuroblastoma in a 47-year-old female with disseminated bone marrow involvement. Bone marrow involvement was the major manifestation which determined the mortality of this patient. This case suggests that the diagnosis of bone marrow involvement should be carefully considered even in adult-onset ganglioneuroblastoma.

[Primary plasma cell leukemia associated with hypercalcemia].

A 34-year-old male was admitted to our hospital because of anorexia and nausea in September, 1987. On admission, anemia, thrombocytopenia and hypercalcemia were observed, and the peripheral blood showed leukocytosis with atypical plasma cells (50%). Bone marrow aspiration showed hypercellularity, with a presence of atypical plasma cells (82%). The M-type protein in the serum and urine was identified as lambda Bence Jones protein by immunoelectrophoresis. On systemic skeletal X-rays, osteolytic lesions were not detected. Thus, a diagnosis of plasma cell leukemia associated with hypercalcemia was made. Hypercalcemia was treated successfully with overhydration. However the disease was refractory to combination chemotherapy, and at 10 months after diagnosis, he died of septic candidiasis. Hypercalcemia associated with plasma cell leukemia in its initial stage is rare.

[A case of fulminant hepatitis after reexposure to halothane six years later].

We reported a case of halothane-induced fulminant hepatitis with acute renal failure which developed 6 days after reexposure to halothane. The patient was a 58-year-old female. She had a history of liver dysfunction after exposure to halothane 6 years previously. She had surgical treatment of clubfoot under halothane anesthesia in other hospital. Preoperative physical examination and laboratory data were normal. On the 6th post-operative day she abruptly developed high fever and general fatigue. Next day, she was transferred to our hospital. At admission, fulminant hepatitis complicated with acute renal failure was diagnosed with severe liver and renal damage. She was immediately treated with plasma exchange, glucose-insulin therapy, and hemodialysis. Serum transaminase level returned to normal value within a week. However, despite repeated hemodialysis, renal function did not improve, and she died of P. aeruginosa sepsis on 28th day after the operation. It may be suggested that in this patient hypersensitivity to halothane has persisted during the six years.