Clinical Reasoning: A 27-year-old man with rapidly progressive coma

Wong, Jonathan; Chandra, Mekhala; Vandebogart, Rachael; Lu, Bo; Yee, Alan

doi:10.1212/wnl.0000000000001887

Cited by 4 publications

(4 citation statements)

References 8 publications

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“…Prenatal diagnosis may be accomplished with DNA analysis of the ornithine transcarbamylase gene obtained via amniocentesis or chorionic villus sampling (1). Treatment involves reducing serum ammonia levels through hemodialysis, dietary protein restriction, and administration of sodium phenyl acetate and sodium benzoate (15).…”

Section: Figurementioning

confidence: 99%

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

2018

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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.

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Section: Figurementioning

confidence: 99%

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

2018

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“…In order to develop standard operating procedures and to define the essentials of an efficient diagnostic work-up, detailed knowledge of the spectrum of pathologies in CUE patients is a prerequisite. Clinicians are aware of this problem and reviews and educational articles have been published on this question [2, 5–7]. However, since their hallmark description of 500 non-traumatic coma patients in an intensive care unit (ICU) by Plum and Posner in the 1970’s [1], only a small number of observational studies on comatose patients from ICUs and EDs have been published [8, 9].…”

Section: Introductionmentioning

confidence: 99%

Causes of brain dysfunction in acute coma: a cohort study of 1027 patients in the emergency department

Schmidt

Ploner

Lutz

et al. 2019

Scand J Trauma Resusc Emerg Med

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BackgroundComa of unknown etiology (CUE) is a major challenge in emergency medicine. CUE is caused by a wide variety of pathologies that require immediate and targeted treatment. However, there is little empirical data guiding rational and efficient management of CUE. We present a detailed investigation on the causes of CUE in patients presenting to the ED of a university hospital.MethodsOne thousand twenty-seven consecutive ED patients with CUE were enrolled. Applying a retrospective observational study design, we analyzed all clinical, laboratory and imaging findings resulting from a standardized emergency work-up of each patient. Following a predefined protocol, we identified main and accessory coma-explaining pathologies and related these with (i.a.) GCS and in-hospital mortality.ResultsOn admission, 854 of the 1027 patients presented with persistent CUE. Their main diagnoses were classified into acute primary brain lesions (39%), primary brain pathologies without acute lesions (25%) and pathologies that affected the brain secondarily (36%). In-hospital mortality associated with persistent CUE amounted to 25%. 33% of patients with persistent CUE presented with more than one coma-explaining pathology. In 173 of the 1027 patients, CUE had already resolved on admission. However, these patients showed a spectrum of main diagnoses similar to persistent CUE and a significant in-hospital mortality of 5%.ConclusionThe data from our cohort show that the spectrum of conditions underlying CUE is broad and may include a surprisingly high number of coincidences of multiple coma-explaining pathologies. This finding has not been reported so far. Thus, significant pathologies may be masked by initial findings and only appear at the end of the diagnostic work-up. Furthermore, even transient CUE showed a significant mortality, thus rendering GCS cutoffs for selection of high- and low-risk patients questionable. Taken together, our data advocate for a standardized diagnostic work-up that should be triggered by the emergency symptom CUE and not by any suspected diagnosis. This standardized routine should always be completed - even when initial coma-explaining diagnoses may seem evident.

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“…Common causes of altered consciousness are substance abuse, medication side effects, trauma, cerebrovascular disease, infections, seizures, and metabolic disturbances [2] .…”

Section: Discussionmentioning

confidence: 99%

“…The magnetic resonance imaging (MRI) finding of the brain diseases is an abnormal signal of cortical with sparing of the perirolandic and occipital cortices [2] .…”

Section: Introductionmentioning

confidence: 99%