2023 American Academy of Neurology Abstract Website

Objective:

Background:

Cushing’s Syndrome (CS) may present with neuropsychiatric manifestations that can mimic autoimmune encephalitis, potentially delaying diagnosis and treatment. We present a case of CS presenting with personality changes and status epilepticus and performed a literature review describing neurological manifestations of CS.

Design/Methods:

A male in his 50s presented with episodic word-finding difficulties, inappropriate smiling, fatigue, cognitive decline and one episode of generalized tonic clonic seizure on levetiracetam from an outside hospital. Workup revealed thrombocytopenia, hypokalemia, hypertension, normal MRI brain and sella. Continuous electroencephalogram revealed nonconvulsive status epilepticus. Lumbar puncture demonstrated elevated protein with normal white blood cell count. He was started on high-dose IV methylprednisolone followed by IVIg for presumed autoimmune encephalitis. Autoimmune encephalitis antibodies including anti-NMDA receptor were negative. CT PET scan showed bilateral adrenal enlargement, which in the context of hypokalemia, hyperglycemia, hypertension, and thrombocytopenia was concerning for severe Cushing’s Syndrome.

Methylprednisolone and anti-seizure medications were stopped. Inferior petrosal sinus sampling and cerebral angiogram were unremarkable. The diagnosis was supported by elevated random and urine cortisol levels and managed with etomidate and ketoconazole. He underwent bilateral open adrenalectomy following which he was started on oral glucocorticoids. Repeat EEG showed no ictal activity and the patient remained seizure-free.

Results:

Literature review utilizing PubMed identified 17 articles describing CS presenting with neuropsychiatric symptoms. The most common symptoms cited were cognitive decline (23%), psychosis (18%) and seizure (6%). Additional isolated cases cited posterior reversible encephalopathy syndrome, hypogeusia and hyposmia. Most patients had significant improvement or full recovery once CS was treated.

Conclusions:

Cushing’s Syndrome can present with neuropsychiatric symptoms and status epilepticus. It can be an emergency requiring prompt endocrine and surgical evaluation. Serum cortisol levels should be checked in patients presenting with acute personality changes and seizures.