An 11-year-old girl with complex seizures was started on valproic acid (VPA) in addition to clonazepam and ethosuximide. Shortly thereafter, she developed marked hyperammonemia that was worsened by a protein load. The hyperammonemia improved somewhat when protein was not given, and it resolved on discontinuation of the valproic acid. No associated changes in serum transaminases or bilirubin were observed. Isolated hyperammonemia may occur soon after VPA ingestion and appears to be a relatively infrequent, reversible side effect. The mechanism of hyperammonemia probably differs from other manifestations of hepatotoxicity, such as elevated transaminases or frank hepatic failure.
Progressive multifocal leukoencephalopathy and malignant lymphoma of the brain were noted at postmortem examination in a 68-year-old white woman who was treated with immunosuppressive agents after renal transplantation. The two diseases are not uncommon in patients with immunodeficiency, but their occurrence in the same patient is extremely rare. This association suggests the oncogenicity of papova viruses in man. However, no papovavirus was demonstrated in the tumor by electron microscopy and immunohistochemical staining. The immunohistochemical staining of routine histology sections for the common antigen of polyomaviruses by the peroxidase anti-peroxidase technique is shown to be simple and specific for the detection of polyomaviruses in the demyelinated areas of progressive multifocal leukoencephalopathy.
A bstract: Somatoform disorders are difficult to diagnosis and often present as a neurological illness in pediatric populations. Conversion disorder is the somatoform disorder most commonly seen in children, particularly adolescents, who have anxiety related to sexual behaviors and orientation. In a transgender patient, the risk of conversion disorder is even higher. The patient described in this article presented with multiple neurological symptoms that disappeared after she began presenting herself as a male. There is a significant need for research into somatoform disorders as well as research into the transgender population. Case StudyMSH was 10 years and 4 months of age when she presented in an emergency room with abnormal jerking movements of the right arm and leg. She earlier had been diagnosed by a psychiatrist with attention deficit/hyperactivity disorder, generalized anxiety disorder, and chronic motor tics, for which she was being treated with mixed salt amphetamine/dextroamphetamine and olanzapine. Her primary care pediatrician had diagnosed her with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS), for which she had been treated with penicillin.During the episodes of right-sided arm and leg jerking, MSH remained alert, awake, and aware of her surroundings. There was no change in her level of consciousness or enuresis during the episodes. The activity was not rhythmic in presentation. Previously conducted tests, including electroencephalograms (EEGs), a magnetic resonance imaging (MRI) scan, and a computed tomography (CT) scan, had revealed no abnormality.MSH's mother reported that approximately 5 days earlier, MSH had started making repetitive guttural noises at school, which had worsened the day before the emergency room visit. The guttural noises had precipitated an altercation with a classmate. MSH stated that she could not make the noises stop, although they completely subsided when she responded to questioning from her teacher. MSH was also having episodes of arm throwing, leg kicking, shivering, slurred or stuttered speech, drooling, regressed primitive language, and hunched posture. Her mother had discontinued the amphetamine salt on her own accord 1 week before a scheduled neurology appointment, but the signs and symptoms had not improved. Increased moodiness, worsening anxiety attacks, and an increased tendency to cry had been noted by the mother since the olanzapine was started.On further questioning, her mother reported that MSH had been having episodes of dizziness for the past 2 years. During the most recent spell, MSH had appeared frightened and pale and had felt nauseated. These events were occurring once a day and were reported to be episodic, without a specific pattern. MSH had experienced severe headaches in the past but not concurrently with the dizziness.Her mother admitted that MSH had some obsessivecompulsive traits. MSH would become extremely upset if her strict, specific routines were not followed. She was fixated on the texture...
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