What’s causing this patient’s spontaneous laughter?

September 19, 2018

Figure 1 Quiz of the Week

A 45-year-old female presents with painful lesions on her lower limbs and ears. She is a smoker, and has a history of recreational drug use. Purpuric, necrotic lesions are noted on both lower extremities, in addition to tender purpura on both ears. A biopsy is performed which demonstrates leukocytoclastic vasculitis.

What’s causing this patient’s spontaneous laughter?

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Image credit: @DrRadioHead


Answer: Precocious puberty

This patient’s clinical presentation and imaging findings are suggestive of a hypothalamic hamartoma, a rare benign malformation that occurs during fetal development. Patients with hypothalamic hamartomas typically present in childhood with refractory gelastic seizures (brief attacks of laughter), precocious puberty, or both. Patients may also develop additional seizure disorders later on, and frequently exhibit developmental delay and behavioral issues.

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