Neuro-Sweet disease presenting as ischemic stroke and asepti
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The present case has been published in the journal Neurology.

• Neuro-Sweet disease (NSD) should be considered in any patient with a history of Sweet syndrome who presents with neurologic symptoms.

• Cerebral infarction can be a manifestation of NSD and may be accompanied by a neutrophilic pleocytosis.

• NSD and its relapses may occur without any dermatologic findings.

• Negative human leukocyte antigen should not deter the clinician from making the diagnosis of NSD, especially in non-Japanese patients.
• NSD may occur in a delayed fashion even after cutaneous Sweet syndrome is treated.

A 59-year-old man with JAK2V617F-positive polycythemia vera (PV), tubulointerstitial nephritis and uveitis syndrome, hypertension, and a remote history of skin biopsy–proven Sweet syndrome presented to the emergency department with 5 days of abnormal gait and diplopia as well as headache, subjective fevers, and diarrhea.

The patient’s medical history was notable for panuveitis with retinal vasculitis diagnosed several years prior to his current presentation. Because of several relapses of his inflammatory eye disease, he was trialed on numerous immunosuppressive medications including cyclosporine, mycophenolate mofetil, and infliximab.

Several weeks after one of his relapses, he developed fever, headache, malaise, arthralgias, night sweats, and a pustular rash on his forearms. Biopsy of the rash revealed a neutrophilic infiltrate and papillary dermal edema without vasculitis, consistent with Sweet syndrome. He was initiated on corticosteroids with improvement.

Although an evaluation for underlying malignancy was initially negative, several years later, he was diagnosed with PV. Despite being maintained on hydroxyurea, he endorsed weight loss and fatigue 1 month prior to admission, raising the concern for uncontrolled PV.

On the day of presentation, the patient awoke with vertical diplopia and gait instability. Upon arrival to the emergency department, he was afebrile and well-appearing. Neurologic examination did not reveal any meningsmus, but was notable for a left hypertropia that worsened with downgaze and rightward gaze (consistent with a left fourth nerve palsy), left hand weakness, and an ataxic gait.

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