To present a rare case of Heerfordt-Waldström syndrome (HWS) featuring atypical neurological symptoms, including trigeminal nerve involvement that resembled an acute stroke presentation.

Heerfordt-Waldström syndrome (HWS) is a rare form of sarcoidosis, characterized by a combination of parotid gland enlargement, uveitis, and facial nerve palsy, often accompanied by fever. While cranial nerve involvement, particularly of the facial nerve, is a well-recognized feature of the syndrome, the involvement of other cranial nerves, such as the trigeminal nerve, is rare. Furthermore, the occurrence of hemibody weakness alongside cranial neuropathies is highly unusual.

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A 45-year-old male admitted to Neurology for right occipital pain, right facial droop, right facial numbness, right-sided upper and lower extremity weakness, photophobia, fevers, and blurry vision. Clinical examination was significant for right parotid swelling, loss of sensation in the right maxillary nerve (V2) distribution, right eye conjunctivitis, axillary lymphadenopathy and subcutaneous nodules, lower motor neuron type facial nerve palsy, right hemiparesis and hemisensory loss, left dilated and unreactive pupil and bitemporal hemianopsia. A stroke workup, including Magnetic resonance imaging (MRI) of the brain, was negative for an acute infarct but showed pituitary macroadenoma and bilateral vestibular schwannoma. Lumbar puncture revealed elevated protein (123 mg/dL). Computer Tomography of the chest and abdomen showed diffuse hilar, axillary lymphadenopathy with splenomegaly and splenic granuloma. Fundoscopy was significant for bilateral retinal granulomas. A cutaneous biopsy showed well-formed, non-caseating granuloma with minimal lymphocytic inflammation of the dermis and subcutis consistent with sarcoidosis. Based on the constellation of symptoms, signs, and biopsy findings, the patient was diagnosed with sarcoidosis and was treated with steroids, plasma exchange, and immunosuppressants.

We present a very rare variant of sarcoidosis presenting with atypical neurologic symptoms treated with a multimodal regimen. This case highlights the atypical neurological manifestations of sarcoidosis and underscores the challenges of diagnosing such unusual presentations.