Cerebellar ataxia and obstructive hydrocephalus, rare neurologic presentations in patients with systemic lupus...

The first cases of systemic lupus erythematosus (SLE) with effects on the nervous system were reported more than 100 years ago. Cerebellar involvement and obstructive hydrocephalus are rarely encountered in patients with SLE. We report two patients with SLE who developed cerebellar ataxia. The first patient presented with significant headache at time of SLE diagnosis and negative brain imaging studies on initial evaluation. The headache recurred with findings of cerebellar ataxia and obstructive hydrocephalus. The patient's symptoms markedly improved with shunt insertion and immunosuppressive therapies. The second patient, known to have SLE, presented with crossed hemiplegia and cerebellar symptoms. Imaging studies showed cerebellar atrophy as well as thalamic and brainstem lesions. The patient had partial improvement with immunosuppression. To understand the impact of SLE on the cerebellum and in hydrocephalus formation, we reviewed the literature and case reports over the last 50 years. Clinical presentations, laboratory results, imaging results, treatment and outcomes, for reported cases found in the literature, were summarized and discussed along with our conclusions. We emphasize the importance of history, examination and close monitoring of patients with SLE presenting with headache or any new neurological symptoms.