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CancerAn Intelligent Diagnosis: SMART Syndrome

An Intelligent Diagnosis: SMART Syndrome

Figure 1Clock-drawing (A) demonstrating left-sided visual neglect prior to receiving methylprednisolone and (B) demonstrating significant improvement after receiving a 5-day course of methylprednisolone.

A 92-year-old woman presented to the Emergency Department with a 10-day history of right-sided headache, confusion, and episodic head turning to the left side that was associated with spontaneous laughter and apparent visual hallucinations. Her family also noted her to be inattentive to her left side. She had been seen in the Emergency Department 3 days prior for the same symptoms, but discharged home after a computed tomography (CT) scan of the head without contrast did not demonstrate any acute abnormalities. Post discharge, her symptoms worsened, which led to her return to the Emergency Department.

Her past medical history was significant for squamous cell carcinoma of the right ear that was treated 9 years prior with radical auriculectomy, cervical lymph node dissection, and local radiation therapy. There was no known recurrence of her cancer. She also had congestive heart failure, paroxysmal atrial fibrillation, coronary artery disease, type II diabetes mellitus, and chronic kidney disease. She had no history of migraine, seizure, or cognitive impairment. Medications included furosemide, ramipril, metoprolol, warfarin, linagliptin, and metformin.

Assessment

On examination, her blood pressure was 162/91 mm Hg, heart rate 84 beats per minute (regular), respiratory rate 18 breaths per minute, oxygen saturation 97% on room air, and temperature 36.7°C. She had evidence of a prior right auriculectomy. She demonstrated marked cognitive slowing and inattention and was witnessed to have recurrent episodes of head turning to the left side that were accompanied by staring and laughter, raising a concern for seizures. On neurologic examination, cranial nerves were normal, although she required cueing in order to direct her gaze to the left. She demonstrated left-sided tactile and visual neglect with a right-sided gaze preference between episodes of head turning. This was further evidenced by her abnormal clock drawing (Figure 1A) and her tendency to only eat food on the right side of her plate. Motor strength was normal. Deep tendon reflexes were brisk in the upper and lower extremities bilaterally. Cerebellar testing and gait examination were limited by confusion.

To read this article in its entirety please visit our website.

-Leora Branfield Day, MD, Phavalan Rajendram, MD, Lorraine V. Kalia, MD, PhD, Wayne L. Gold, MD

This article originally appeared in the January 2021 issue of The American Journal of Medicine.

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