A 29-month-old girl with worsening ataxia, nystagmus, and subsequent opsoclonus and myoclonus

Article Abstract:

A 29-month-old girl with ataxia, irregular eye movement, and vomiting was found to have opsoclonus-myoclonus-ataxia syndrome with an adrenal gland tumor. Opsoclonus-myoclonus-ataxia syndrome is a movement disorder that is often caused when the immune system responds initially to a tumor and then attacks the nervous system. The child had difficulty walking, a tremor in her hands and arms, and poor muscle tone. X-rays, CT scans, ultrasound and magnetic resonance imaging showed slight enlargement of the brain ventricles and an adrenal tumor. The tumor was surgically removed and found to be a ganglioneuroblastoma. Following surgery and corticotropin therapy the muscle and eye spasms subsided but the girl only spoke single words. Genetic aspects of the tumor suggested a favorable prognosis, however the persistence of neurologic problems is unfavorable.

Ataxia, Neuroblastoma, Tumors in children, Pediatric tumors, Myoclonus, Nystagmus

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Progressive neurodegenerative disease in a young boy

Article Abstract:

A five-year-old boy died from a progressive neurodegenerative disease. Abnormalities were first noticed when he was 12 months old, and the child had been examined by a neurologist when he was 16 months old. He had experienced muscle spasms and seizures. His motor and verbal abilities had gradually deteriorated, and he had been bedridden since the age of four. Magnetic resonance imaging (MRI) scans showed that parts of his brain had atrophied, and the results of laboratory tests were abnormal. An autopsy revealed a brain that was smaller than normal with major abnormalities. His muscles were smaller than normal, and his liver was enlarged. A younger sister had a similar neurodegenerative disorder. The patient was diagnosed with an inherited neurodegenerative disease: infantile striatonigral degeneration with cerebellar degeneration.

Nervous system, Nerve degeneration, Nervous system diseases

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Case 36-2005: A 61-year-old woman with seizure, distributed gait, and altered mental status

Article Abstract:

A 61-year-old woman was admitted to the neurology service of a hospital due to a seizure anA 61-year-old woman was admitted to the neurology service of a hospital due to a seizure and altered mental status. She was diagnosed with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes.

United States, Usage, Diagnosis, Neurologic manifestations of general diseases, Neurologic manifestations, Gait disorders, Biopsy

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