Polimiositis y dermatomiositis asociadas con neoplasias: estudio retrospectivo de 30 años / Polymiositis and dermatomyositis associated with malignancy: a 30-year retrospective study

La polimiositis y la dermatomiositis asociadas con neoplasias son síndromes paraneoplásicos, pero la incidencia, el tratamiento y los factores que predicen el cáncer asociado a estas enfermedades y su pronóstico no están muy claros. Pacientes y Métodos: Durante el período de 30 años desde 1969 a 1999 tratamos a 64 pacientes que tenían polimiositis (dos con cáncer) y 28 pacientes que tenían dermatomiositis (10 con cáncer). Comparamos los resultados clínicos de los pacientes que tenían cáncer con los de los que no los tenían. Resultados: El riesgo de cáncer es significativamente mayor en la dermatomiositis y algo mayor en la polimiositis. Se encontró un mayor riesgo de cáncer en varones con dermatomiositis mayores de 50 años. El cáncer se diagnóstico a lo largo de cuatro años antes o después del diagnóstico de la polimiositis o de la dermatomiositis y, generalmente, en un año. No fue posible intervenir a muchos de los pacientes con cáncer debido al estadio avanzado de la enfermedad. Conclusión: Nuestros resultados indican que el descubrimiento precoz del cáncer es crítico en casos de polimiositis y dermatomiositis (AU)

Bupivacaine hydrochloride induces muscle fiber necrosis and hydroxyl radical formation-dimethyl sulphoxide reduces hydroxyl radical formation.

We induced acute skeletal muscle necrosis in rats using bupivacaine hydrochloride and found that both 2,5- and 2,3-dihydroxybenzoic acid significantly increased in skeletal muscle. A single administration of dimethyl sulphoxide, a free radical scavenger, significantly lowered concentrations of 2,5- and 2,3-dihydroxybenzoic acid. These results suggest that dimethyl sulphoxide is an effective hydroxyl radical scavenger and may be useful in the treatment of myopathy.

[A thyrotoxic myopathy accompanied with unusual muscle symptoms and MRI muscle findings].

We report a patient with thyrotoxic myopathy associated with unusual muscle symptoms. A 29-year-old man developed hyperhidrosis, diarrhea, increase in appetite, and excitability in July, 1999. In August, he experienced muscle stiffness in bilateral lower extremities after maintaining postures such as driving a car or sitting on a chair. He was admitted to our hospital, in January, 2000. On physical examination, goiter was noted. Neurological examination was normal except for proximal muscle weakness. Laboratory test showed elevated free T3 and free T4, decreased TSH. TSH receptor antibody was increased. MRI of lower extremities revealed atrophy of bilateral biceps femoris. Muscle strength increased gradually after an oral administration of thiamazole 30 mg/day, and muscle stiffness disappeared. The clinical features of this patient and differential diagnosis were discussed.

IDPN-induced monoamine and hydroxyl radical changes in the rat brain.

beta-beta'-iminodipropionitrile (IDPN)-induced monoamine and hydroxyl radical changes in the rat brains were studied. IDPN caused decreases in 5-HT and 5-HIAA levels in all brain regions, strongly indicating that IDPN's neurotoxicity primarily affects 5-HT containing neurons. Dopamine and its metabolites' levels decreased in the some regions, most likely due to depression of dopamine metabolic turnover. Our results more clearly demonstrate IDPN-induced monoamine alterations in the rat brain more than previous reports. To clarify one of the pathogenesis of IDPN-induced neurological disorders, we measured hydroxyl radical levels. 2,3-DHBA increased at 1st day, and decreased in some regions at 7th days after discontinuing IDPN. We conclude, hydroxyl radical formation causes neuronal damage, and monoamine changes contribute to IDPN-induced neurological disorder.

[Treatment of myasthenia gravis].

The strategy of myasthenia gravis has been progressed during recent 30 years. Extended thymectomy and alternate-day prednisolone administration remarkably improved therapeutic effect in myasthenia gravis. Besides of these therapies, plasmapheresis and immunoadsorption plasmapheresis extremely improved mortality in myasthenia gravis. 1. Corticosteroid therapy Usually initial doses of PSL is 20-30 mg every other day in the morning after thymectomy, and increased 5 mg every on-day. Maximum PSL doses is 80-100 mg alternate-day, then this doses will be maintained over 4 weeks. After these procedures, PSL is gyradually decreased and discontinued within 2-3 years. 2. Thymectomy Extended transsternal thymectomy is the most useful method, because many authors have reported that re-thymectomy reveals re-appearance of thymus. 3. Invasive thymoma Although this type of thymoma has been called malignant thymoma, it should be called as invasive thymoma since any thymoma can invade surrounding tissues for a long subclinical period. Strategy of treating invasive thymoma is PSL administration, radiation and chemotherapy. Chemotherapy should be performed depend on histopathological findings of thymoma. CHOP therapy is useful for the lymphocytic cell predominant type of thymoma, however CHOP + cis-platin therapy is useful for the epithelial cell predominant type. Treatment of myasthenia gravis has been improved, however it is still unclear that how to treat invasive thymoma, and how to prevent re-appearance of symptoms by decrease or discontinuity of PSL administration.

[Sensory disturbance of crossed oral-pedal topography in a case of lateral medullary infarction].

We report a 40-year-old man with hypertension and diabetes mellitus, who had crossed oral-pedal sensory disturbance in lateral medullary infarction. He suddenly developed dysesthesia in the right mount and the left leg. His blood pressure was 150/90 mmHg. Neurological examination showed Horner's sign in the right eye and horizontal nystagmus. Sensory function revealed decreased temperature, hypalgesia and dysesthesia in the right mouth and the left leg. Vibratory and position sense were normal. T1- and T2-weighted images disclosed a low and high signal intensity area in the lateral portion of the right medulla oblongata, respectively. Brain and neck MRA using time-of-flight sequence revealed no obvious abnormal structures. We have diagnosed him as lateral medullary infarction. The unique topography of sensory dysfunction thought to be attributed to a far-lateral lesion in the medulla oblongata. Our patient suggests that lateral medullary infarction causes variable patterns of sensory disturbance. Thus, lateral medullary infarction should be warranted when we encounter patients with miscellaneous distribution of sensory impairment, such as crossed mouth-foot hypalgesia.

Satoyoshi's syndrome in an adult: a review of the literature of adult onset cases.

Satoyoshi syndrome consists of painful intermittent muscle spasms, alopecia and diarrhea. The age of onset is commonly less than 20 years of age. Adult onset of this syndrome is rare. We report a 65-year-old Japanese woman with Satoyoshi's syndrome of adult onset. Satoyoshi's syndrome of adult onset shows no skeletal abnormalities, in comparison with the classical pediatric cases. The clinical features in our patient revealed the benign and long-term course of muscle spasm and alopecia. This progression differed markedly from the other patients of adult onset. Thus, the severity of Satoyoshi's syndrome is variable in adult onset cases, and this diagnosis should be considered in adults with various and unexplained muscle spasms.

[A case of eosinophilic meningoencephalitis caused by Angiostrongylus cantonensis with unique brain MRI findings].

A 17-year-old Japanese girl was admitted to our hospital because of meningeal irritation, hyperesthesia of extremities, and disturbance of consciousness after having traveled to Okinawa in February, 1996. Examination on admission revealed pleocytosis of CSF with many eosinophils (65%) and increased eosinophils (25%) in the peripheral blood. The history and clinical symptoms were suggestive of a parasitic infection. We diagnosed the patient as having eosinophilic meningoencephalitis caused by Angiostrongylus cantonensis, which was confirmed by immunological tests of double diffusion, counterimmuno-electrophoresis, and ELISA. Magnetic resonance imaging (MRI) of the brain demonstrated multiple small high intensity areas on Gd-DTPA-enhanced T1-weighted images. MRI findings suggested tissue reactions to dead or dying worms, and local vasodilatation associated with minimal thrombus formation.

[Polymyositis after interferon beta treatment of chronic hepatitis type C].

We reported a case of polymyositis manifested after beta-interferon therapy for chronic viral hepatitis type C. In November 1992, a 62-year-old female was diagnosed as having chronic viral hepatitis type C. In January 1993, she was treated with beta-interferon(total 2.52 x 10(8)IU). In March 1993, after the treatment, she noticed weakness and atrophy of her lower extremities. Neurological examination revealed proximal dominant muscle weakness and atrophy of the extremities. Increased levels of serum CK and aldolase were disclosed on admission. By needle EMG, a low voltage with short duration interference pattern was noted. February 7, by muscle biopsy of the right quadriceps, necrotic myofibers, marked variation in fiber diameter, stromal mononuclear cell infiltration, and endomysial fibrosis was detected. The immunological stain of infiltrating cells in the muscle fibers revealed CD4:CD8 ratio as 64:55, whereas in conventional polymyositis CD8 cells dominate CD4 cells. Diagnosis of polymyositis was made and oral prednisolone 60 mg a day was started. Her symptoms and signs improved gradually and she was discharged after 2 months. Many adverse effects due to immunological disturbances caused by interferon treatment have been reported, but polymyositis due to interferon has been very rare. When interferon will be prescribed, manifestation of polymyositis should be considered.

A case of myasthenia gravis accompanied by invasive thymoma, alopecia areata and dry mouth.

We report a 43-year-old man suffering from myasthenia gravis with invasive thymoma accompanied with alopecia areata and dry mouth. These complications are extremely rare and the pathogenetic etiology of these complications was thought to depend on a generalized immunological disturbance.

Tethered cord syndrome accompanied by unilateral muscle atrophy in the calf muscle.

A 40-year-old man developed slowly progressive muscle atrophy in his calf muscle. The patient became aware of asymmetry of his right big toe during in his teens; muscle atrophy was revealed at the age of 25 years. Multi-image examinations revealed "tethered cord syndrome" with spina bifida occulta. Spinal magnetic resonance image revealed a pathogenetic mechanism of asymmetry of muscle atrophy in the calf muscle. This suggested that spinal MRI imaging is one of the most useful diagnostic methods for this disease.

Myasthenia gravis and invasive thymoma. A 20-year experience.

Over the last 20 years, 15 patients with myasthenia gravis and invasive thymoma have been treated in our department. Eight of these patients underwent either nontotal excision or were not operated on due to massive invasion, while 7 underwent total excision. These 15 cases represent 6.2% of the 242 myasthenic patients treated during this period. Six of the 8 nontotal excision cases died, and 2 of the 7 total excision cases succumbed. The prognosis of total excision cases was better than that of nontotal excision cases. The overall 5-year survival rate was 73%, and the 10-year survival rate 20%. Total excision of the thymoma, if possible, and high doses of corticosteroids and combination chemotherapy for the remaining tumor seem to be the treatments of choice.

Multiple liver abscesses secondary to Yersinia enterocolitica.

A 37-year-old male, a poorly-controlled insulin-dependent diabetic patient, was admitted to our hospital with complaints of high fever and confusion. Laboratory data showed hyperglycemia, positive inflammatory reaction and liver dysfunction. Blood culture demonstrated Yersinia enterocolitica. Liver CT scan showed multiple low density areas. These data were consistent with a diagnosis of liver abscess secondary to Yersinia enterocolitica. He died of disseminated intravascular coagulation; subsequent autopsy confirmed the clinical diagnosis. Liver abscess secondary to Yersinia enterocolitica with septicemia is rare, but has been reported in compromised hosts. In the mechanism of this disease, the alimentary tract has been suggested to be the port of entry in most cases.

Biochemical study of different conditions of type 2C muscle fiber in rat.

Type 2C muscle fibers were histochemically and biochemically examined under three different conditions in rats: in the neonatal stage, after neonatal denervation and on regenerating process after bupivacaine-induced injury. On histochemical examination, soleus was found to contain up to 90% of type 2C fiber in every condition, whereas both gastrocnemius and extensor digitorum longus contained 40-70%. On biochemical analysis, triglyceride was the smallest and glycogen was the largest in amounts in neonatal muscles, triglyceride was the largest and glycogen was lowest in amount in neonatal denervated muscles, while both triglyceride and glycogen were low in amounts in bupivacaine-treated muscles. These results suggested that although histochemical characteristics are the same, significant differences in biochemical properties exist between type 2C fibers in these three different conditions.

[A case of carcinomatous autonomic and sensory neuropathy].

A 61-year-old male with carcinomatous autonomic and sensory neuropathy was presented. The disease started with numbness in his lower extremities and loss of sensation gradually developed over the upper extremities within two months. Finally he became unable to walk because of orthostatic hypotension and dystaxia due to loss of deep sensation. Physical examination on admission revealed mydriasis with negative light reflex, orthostatic hypotension, sensory loss of below C-4, areflexia, positive Babinski's sign and sexual impotence. Laboratory examinations revealed serum antineural antibody, elevation of protein and oligoclonal IgG band in the CSF. Sural nerve biopsy specimen showed severe loss of myelinated fibers, especially in the large-sized fibers. The pharmacological functional tests of the autonomic nerves disclosed disturbance of parasympathetic nervous system as well as the postsynaptic fibers of the sympathetic nerves. An instillation test of 2.5% mecholyl solution demonstrated contraction of both pupils. Chest X-ray showed abnormal shadow at the right hilus and transbronchial biopsy revealed small cell carcinoma (oat cell type). Plasma exchange improved orthostatic hypotension and decreased the titer of the antineural antibody. In this case, the autonomic ganglion including the ciliary ganglia and the dorsal root ganglia cells were predominantly involved and such cases have been described as a variant of paraneoplastic syndrome in the literature.