Case 252: Acute Hyperammonemic Encephalopathy Resulting from Late-Onset Ornithine Transcarbamylase Deficiency.

History A 19-year-old woman with no pertinent medical history was brought to the emergency department after being found unconscious on her bathroom floor by her roommate. In the preceding weeks, she had reported intractable nausea and vomiting, for which she had been taking ondansetron. No other medications had been prescribed. The day prior to presentation, she had contacted her mother and described increasing confusion. Glasgow coma scale score on arrival in the emergency department was 4. Intravenous naloxone was administered, without immediate response. Initial blood glucose level was 232 mg/dL (12.8 mmol/L) (normal range, 79-140 mg/dL [4.4- 7.7 mmol/L]), and other routine laboratory test results were normal. Urine toxicology results were negative. Cerebrospinal fluid evaluation revealed levels were within normal limits. Neurologic examination revealed dilated pupils, which showed a sluggish response to light, and left lower extremity rigidity with intermittent tremors. Initial unenhanced cranial computed tomographic (CT) findings were negative. Magnetic resonance (MR) imaging of the brain was performed. The patient's condition deteriorated, with increasing cerebral edema over the next week, and she was declared brain dead. Her liver was transplanted into an adult recipient, who subsequently developed cerebral edema and elevated plasma ammonia levels, resulting in death in the immediate postoperative period.

The First Reported Case of Recurrent Carcinoid Tumor in the External Auditory Canal.

OBJECTIVE: Cutaneous neuroendocrine lesions of the external auditory canal (EAC) are exceptionally rare, with only five cases reported in the literature. In this case report, we present a patient with a recurrent carcinoid tumor in the EAC, which has yet to be described. PATIENTS: A 38-year-old woman presenting with otalgia, aural fullness, and decreased hearing was found to have a recurrent EAC carcinoid tumor, 8-years after initial resection at an outside facility. INTERVENTIONS: The recurrent tumor involved much of the proximal, anterior bony ear canal and was trans-tympanic, extending to the middle ear and epitympanum; therefore, a lateral temporal bone resection was performed to ensure complete resection. MAIN OUTCOME MEASURES: Surgical pathology confirmed the presence a recurrent carcinoid tumor in the EAC, with immunohistochemistry positive for pancytokeratin (MAK6), CD56, and synaptophysin, with chromogranin showing rare cells positive for cytoplasmic granules. There was no evidence of metastasis. RESULTS: Lateral temporal bone resection was successful and the patient is being followed with annual imaging. The patient is considering future hearing rehabilitation with a bone anchored hearing device. CONCLUSION: This case report highlights the first known case of recurrent carcinoid tumor in the EAC, treated with lateral temporal bone resection. Clinical presentation, imaging, treatment, and pathology are reviewed along with a review of the literature.

Evaluation of emergency CT scans of the head: is there a community standard?

OBJECTIVE: This study was designed to assess the accuracy of general radiologists in the interpretation via teleradiology of emergency CT scans of the head. MATERIALS AND METHODS: We studied the interpretations of 716 consecutive emergency CT scans of the head by a group of 15 board-certified general radiologists practicing in the community (as opposed to an academic setting). The scans were sent via teleradiology, and the preliminary interpretations were made. Three of the general radiologists were functioning as nighthawks, and the remaining 12 were acting as on-call radiologists in addition to their normal daytime duties. Each CT examination was interpreted by one of five neuroradiologists the day after the initial interpretation had been performed. The findings of the final interpretation and the preliminary interpretation were categorized as showing agreement, insignificant disagreement, or significant disagreement. The reports in the two categories indicating disagreement were reviewed and reclassified by a consensus of three university-based neuroradiologists. RESULTS: Agreement between the initial interpretation by the general radiologist and the final interpretation by the neuroradiologist was found in 95% of the CT scans. The interpretations were judged to show insignificant disagreement in 3% (23/716) of the scans and to show significant disagreement in 2% (16/716). Of the 16 significant errors, five were false-positive findings and 11 were false-negative findings. Forty-seven CT scans depicted significant or active disease, and in 11 (23%) of these scans, the final report differed significantly from the preliminary interpretation. Three patients had pituitary masses, none of which had been described on the preliminary interpretation. CONCLUSION: The rate of significant discordance between board-certified on-call general radiologists and neuroradiologists in the interpretation of emergency CT scans was 2%, which was comparable to previously published reports of residents' performance. The pituitary gland may be a blind spot, and additional attention should be focused on this area.