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May Case of the Month

Clinical History:

43-year-old male with a chronic history of dysesthesias and hypesthesias in all extremities. He has a chronic disease of which the most important findings are liver failure and decreased vision. Several members of his family had a similar history.

View Diagnosis

Primary Diagnosis

Familial amyloid polyneuropathy.

Secondary Diagnosis

spinal canal, spinal cord, subarachnoid space

Diagnosis Discussion

Familial amyloid polyneuropathy: Deposition of amyloid in pial/subarachnoid arteries & arterioles results in destruction of the blood-cord-barrier sensory nerves & unmyelinated fibers most affected Precursors of FAP are metabolized by liver, thus liver transplantation is useful. Amyloidosis of the spinal cord predominantly involves the arteries in the subarachnoid space

General Anatomic Area:

Other

Secondary Anatomic Area:

spinal canal, spinal cord, subarachnoid space

Disease Category:

Metabolic

Contributor:

Maurice Castillo, MD

Date:

05/01/2005

Difficulty Level:

Difficult

Pathology Confirmed?:

Yes
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ACR Category Number:

N/A

Keywords:

N/A