7/10/2004 Case 1: Chronic inflammatory demyelinating peripheral neuropathy

Clinical History:

This was from a 14 year old girl who had no significant medical history until she presented at age 9 with "right optic neuritis". She recovered fully without treatment and was lost to follow up. A year later she presented with right hemiparesis, and CT and MRI, plus evoked potential studies, were all consistent with a diagnosis of MS . Again she recovered completely with no treatment. Since that time she has had several episodes requiring hospital admission due to weakness involving one or another extremity; these have resolved following treatment with IV Solumedrol; however she was found to have absent deep tendon reflexes and her weakness was predominantly distal, prompting a sural nerve biopsy. Dr Rao distributed 2 x 2 projection slides of the nerve biopsy, with one H&E image and one trichrome stain.

Diagnostic Notes:

These show nerve with perivascular and intra-endoneurial mononuclear cell inflammatory infiltrates. There is a marked loss of myelin (essentially there is none, but there is myelin debris apparent in a myelin stain shown at the conference). There are multiple digestion chambers of Cajal . There are no onion bulbs; a Bielschowsky stain shows a considerabte loss of axons
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Slide Image:

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