Neuro-Sweet Syndrome: A Diagnostic Conundrum

Area of Science:

• Neurology
• Dermatology
• Immunology

Background:

• Sweet Syndrome (acute febrile neutrophilic dermatosis) typically involves skin and systemic symptoms.
• Neurological involvement in Sweet Syndrome is rare but can present as encephalopathy.
• Differentiating neuro-Sweet Syndrome from other encephalitic conditions is challenging due to varied presentations.

Observation:

• A 51-year-old female presented with fever, rash, headache, and encephalopathy.
• Brain MRI revealed extensive T2 hyperintensities in the cerebral hemispheres, cerebellum, and brainstem.
• Skin biopsy confirmed dermal neutrophilic infiltration, consistent with Sweet Syndrome.

Findings:

• The patient responded well to steroid treatment, suggesting an inflammatory etiology.
• A history of a similar episode 10 years prior, misdiagnosed as acute disseminated encephalomyelitis, was uncovered.
• This case highlights the potential for relapsing neuro-Sweet Syndrome over extended periods.

Implications:

• Neuro-Sweet Syndrome should be considered in cases of acute encephalitis with white matter lesions, especially when steroid-responsive.
• High clinical suspicion is necessary for diagnosing neuro-Sweet Syndrome, particularly with a history of recurrent neurological episodes.
• Recognizing neuro-Sweet Syndrome aids in timely and appropriate treatment, potentially preventing misdiagnosis and delayed management.