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Abstract Details

Reversible Splenial Lesion Syndrome (RESLES) in Sickle Cell Crisis Mimicking Marchiafava-Bignami Disease
Neuro Trauma and Critical Care
P10 - Poster Session 10 (8:00 AM-9:00 AM)
1-001

Reversible Splenial Lesion Syndrome (RESLES) is characterized by the presence of transient lesion involving the splenium of corpus callosum in patients with encephalopathies of various cytotoxic etiologies. One of which is Marchiafava-Bignami Disease(MBD), which could manifest with sudden stupor or coma in alcohol use disorder (AUD) or malnutrition with severe demyelination of splenium. Here we report a case of MBD-like presentation in a patient with vaso-occlusive sickle-cell crises. 

 

 

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A 46-year-old male with sickle-cell disease and no history of AUD presented with acute chest syndrome with pain crisis. He was treated with blood transfusion and analgesics. Two days later, he became acutely unresponsive with right-sided hemiparesis, requiring intubation. MRI brain showed numerous punctate infarcts in the watershed areas bilaterally, along with symmetric diffusion restriction in the splenium of corpus callosum, respecting its anatomic boundary. Exchange transfusion was initiated and maintained his hemoglobin >10 g/dL and Hb-SS < 30%, but he remained comatose (GCS 6). Electroencephalogram showed diffuse cerebral dysfunction without epileptogenicity. His extensive infectious workup was unremarkable. Other laboratory tests including electrolytes, liver function tests, ammonia, thyroid function tests, vitamin B12, B6, folic acid, and thiamine levels were normal. He was empirically treated with intravenous thiamine. One week later, his mental status improved to following commands. MRI brain two weeks after showed a near-complete resolution of the callosal lesion. The patient’s acute stupor/coma and MRI findings were attributed to hypoxic watershed infarcts and RESLES due to hypoxic stress from vaso-occlusive sickle cell crisis, leading to a transient coma, mimicking MBD. 

RESLES is a distinct syndrome with characteristic radiographic finding of diffusion restriction in the splenium of corpus callosum due to cytotoxic edema of various etiologies. MBD is one such example, presenting with stupor/coma. This case emphasizes the importance of identifying reversible metabolic stressors, including sickle cell crisis. 

Authors/Disclosures
Yohannes Simeneh Mulatu, MD (SUNY Downstate)
PRESENTER
Dr. Mulatu has nothing to disclose.
Joseph YoungHun Yoon, MD (Mount Sinai ) Dr. Yoon has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ECHAS.
Srinath Ramaswamy, MD (SUNY Health Science Center, Department of Neurology) Dr. Ramaswamy has nothing to disclose.
Biniyam Simeneh Mulatu, MD (Addis Ababa University) Dr. Mulatu has nothing to disclose.
Cappi C Lay Cappi C Lay has received publishing royalties from a publication relating to health care.