We describe three patients with mitochondrial myopathy, dementia, loss of vision and hearing, seizure disorder with myoclonus, intermittent headaches of a vascular type, visual hallucinations, cerebellar dysfunction, and lactic acidosis. Muscle biopsies in all patients and liver biopsy in one revealed abnormal mitochondria. The disorder may be due to a deficiency of mitochondrial NADH-CoQ dehydrogenase.
Several distinct neurological complications have been described in Legionnaires' disease. These include: encephalopathy [4], encephalitis with prominent brainstem signs [7], cerebellar involvement [ 5 , 7, 91, myelopathy [ l , 21, and peripheral nerve involvement [3, 6, 81. Of the seven patients with documented cases of peripheral neuropathy associated with Legionnaires' disease, four were asymptomatic and were diagnosed by abnormalities in electromyography and nerve conduction velocities. The other three patients had clinical signs of neuropathy that developed during the course of the disease and later resolved almost completely. We report a patient with an acute, profound motor neuropathy with onset that preceded the development of respiratory signs of Legionnaires' disease. There has been only slight improvement in the neuropathy in more than seven months of follow-up care.A previously healthy 61-year-old man with a history of chronic ethanol abuse and chronic obstructive lung disease developed diarrhea and generalized myalgia, followed three days later by weakness of the left hand grip. Muscle weakness developed rapidly, and the patient became nearly quadriplegic within 16 hours. When he was examined in the emergency room, blood pressure was 140/80, pulse 80 per minute, respirations 16 per minute, and temperature 38.65"C. The general physical examination was normal. O n neurological examination the patient was alert and oriented, speech was intact, and mentation was normal. The cranial nerves were intact. Examination of the motor system revealed a complete flaccid quadriplegia, only slight movement remaining in the digits of both legs and in the right arm. Sensation was intact, and stretch reflexes were depressed but absent at the ankles. There was a bilateral flexor plantar response and no nuchal rigidity. Tidal volume and vital capacity were within normal limits. Three hours after the initial examination, the patient was areflexic and could move only the second and third digits of his right foot very minimally. Blood counts, blood chemical screen, and urinalysis were normal, as was the chest roentgenogram. The lumbar puncture was traumatic but resolved to a clear fluid under normal pressure. The cell count showed 8 white blood cells (6 mononuclear, 2 polymorphonuclear) and 6,620 red cells per cubic millimeter, glucose 83 mg/dl, and a protein content of 26 mg/dl.The patient was treated with 60 mg of prednisone daily. Two days later, myelography performed in the prone position revealed no abnormalities. A chest roentgenogram taken at this time showed a right lower lobe pneumonia. Cell count revealed 18,000 leukocytes per cubic millimeter with a left shift. The patient became dyspneic and required intubation. Emergency bronchoscopy showed purulent material in the right mainstem bronchus. The following day, bronchial washings taken at the time of bronchoscopy were reported to show a positive immunofluorescent stain for Legionella pneumophilia, group 6. The patient was subsequently treated successfully with ...
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