Adverse neurologic effects of glucocorticosteroids.
Explore the source record for details and available documents.
Adolescent↗
Biomedical subjects
D Lacomis
Publications and source records attributed to D Lacomis.
Childhood onset oculopharyngeal muscular dystrophy.
Oculopharyngeal muscular dystrophy is an inherited disorder, usually autosomal dominant, which typically becomes symptomatic during the fifth decade of life with slowly progressive ptosis and dysphagia; childhood onset has not been reported. A 13-year-old female of French-Canadian descent developed nasal speech and strabismus at 5 years of age; there was no family history of neuromuscular disease. Ptosis and mild facial and proximal muscle weakness were present by 9 years of age. Over the next 4 years, the patient developed dysphagia, palatal paralysis, weight loss, decreased ocular motility, scoliosis, shortness of breath, and obstructive apnea. Tracheostomy and gastrostomy were required. Creatine kinase and repetitive facial nerve stimulation were normal. Edrophonium testing was negative and electromyography revealed myopathic motor units in the iliopsoas muscle. A preponderance of type I fibers and scattered atrophic and angulated muscle fibers were present in 3 muscle biopsies. The clinical presentation and findings are consistent with childhood onset oculopharyngeal muscular dystrophy.
Adolescent↗
MR imaging of paraneoplastic limbic encephalitis.
Paraneoplastic limbic encephalitis is a rare disorder that has been previously diagnosed on clinical and pathologic grounds without good radiologic correlation. We present the case of a 42-year-old woman who developed gradually progressive limbic dysfunction 4 years after undergoing mastectomy for breast cancer. Although CT scans were normal, magnetic resonance (MR) imaging showed signal abnormalities in the medial portions of both temporal lobes, the amygdaloid nuclei, and the hypothalamus. An MR-guided temporal lobe biopsy confirmed the presence of encephalitis.
Adult↗