Guest Post from Howard Chang, MD, PhD: Unknown White Matter Disease

I am please to present a guest post from the illustriousDr. Howard Chang of Michigan State University, who presents a perplexing case. Dr. Chang would be interested in reader comments. He writes: "I can use some help from our colleagues.  Any advice (from anyone) on where and how to proceed for additional studies will be very much appreciated. "Dr. Howard ChangThis is a case of a 12-year-old male with cerebral palsy, severe developmental delay (level 1-2 years), and seizures (stable, no seizure episodes since 2 years). He had progressive decline in neurological functions following flu-like illness. He received IVIG and steroids for clinical diagnosis of GBS-CIDP (18 months prior to death). Initially he showed some improvement, but neurological functions continued to decline, with multiple hospitalizations.  MRI imaging studies (2 weeks prior to death) showed extensive abnormal signal of the cerebral and spinal white matter. He was made DNR. A general autopsy including brain and spinal cord was performed.General Autopsy:1. Atrophy of low extremity muscles and apparent atrophy of muscles of hands.2. Cushingoid appearance with central obesity, skin striations, and adrenocortical atrophy (likely due to steroid therapy).Neuropathology Autopsy:1. Extensive white matter atrophy-degeneration involving both the brain and spinal cord (leukoencephalomyelopathy) with:A. Microcephalic brain (weight 1050 gm, normal should be about 1400 gm).B. Bilateral cerebral white matter atrop...
Source: neuropathology blog - Category: Radiology Tags: neuropathologists Source Type: blogs