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A woman in her 60s presented to the emergency department with nausea, vomiting and abdominal pain for 7 days. Two days later she developed progressively worsening dysphagia to both solids and liquids.
Initial investigations showed a mild leucocytosis (11.6×109/L) with neutrophilia (8.88×109/L). Serum C-reactive protein was elevated at 21 mg/L (<10). Other blood tests, including liver, renal and thyroid function were normal. Flexible nasal endoscopy showed pooling of mucus in the throat and an inability to swallow. The vocal cords and piriform fossae appeared normal. A non-contrast CT scan of the neck showed no definite laryngeal or upper airway lesions.
48 hours after admission, the patient became dyspnoeic and required supplemental oxygen to maintain her oxygen saturation. She was noted to have a lower-pitched voice. Shortly thereafter, she became confused and suffered a respiratory arrest. She received cardiopulmonary resuscitation with the return of spontaneous circulation after 2 min. She was intubated, ventilated and transferred to the intensive care unit. She had no dysarthria, dry mouth, blurred vision or diplopia.
Her initial neurology evaluation was while she was sedated and intubated, limiting the assessment. Pupils were bilaterally fixed and dilated, deep tendon reflexes were reduced. Plantar responses were extensor on the left and withdrawal on the right. A CT scan of the head showed no acute intracranial pathology. MR scan of the brain could not be performed owing to her being intubated. A contrast-enhanced CT scan of the neck was degraded by streak artefacts making it diagnostically suboptimal. A contrast-enhanced CT scan of the thorax showed the oesophagus was mildly dilated proximally but collapsed distally. Video fluoroscopy showed mild tongue weakness and slow transit through the upper oesophagus, indicating oesophageal dysmotility probably from autonomic dysfunction.
Re-examination after stopping sedation showed bilateral asymmetrical ptosis. Pupils were fixed and dilated, with a …
Footnotes
Contributors All authors and contributors have agreed for the manuscript. TH, Consultant Neurologist, Kettering General Hospital will be the guarantor.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned. Externally peer reviewed by Jon Walters, Swansea, UK.