Abstract Details

Title AL-Amyloidosis presenting with painful mononeuropathy multiplex and bilateral cranial nerve 3 palsies

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P12 - Poster Session 12 (12:00 PM-1:00 PM)

Poster/Presentation
Number 1-005

Objective To describe the clinical and pathological findings of a rare presentation of AL-amyloidosis.

Background AL-amyloidosis is the most common cause of systemic amyloidosis, where abnormal light chains are deposited within various organs. Neurological involvement is typically an axonal peripheral and autonomic neuropathy.

Design/Methods N/A.

Results A 74-year-old woman with type 2 diabetes initially presented with a 9-month history of bilateral burning hand paresthesias. One month later she developed diplopia, followed by stepwise and painful weakness of right then left finger flexors, then weeks later, right wrist and finger extensors. Examination showed bilateral fixed and mid-dilated pupils, ptosis, proptosis and limited bilateral eye elevation, depression and adduction. There was bilateral hand muscle atrophy with asymmetric (right>left) weakness of finger flexors, abductors, and extensors, and diffuse loss of pinprick sensation in the hands. Reflexes were reduced in the upper limbs. Strength, reflexes and sensation were normal in the legs. Electromyography was consistent with a post-ganglionic axonal peripheral neuropathy of the upper limbs. Brain and spine MRI showed an incidental pituitary adenoma and multifocal areas of enhancement within the cauda equina. Cerebrospinal fluid showed mildly elevated protein only. Bloodwork revealed mild anemia and positive anti-PR3. CT scans showed multiple enlarged lymph nodes and enlargement of the appendix. Bone marrow biopsy showed no lymphoproliferative disorder. Cardiac MRI was normal. Appendectomy and lymph node biopsy showed extensive amyloid deposition, and mass spectrometry confirmed kappa-light-chain amyloid. The patient was treated with prednisone, cyclophosphamide and pomalidomide, but due to weight loss, anorexia and vomiting, medications were stopped. Further follow-up will be presented at the time of the conference, but there was some consideration of a palliative route.

Conclusions

AL-amyloidosis has very rarely been reported presenting with mononeurpathy multiplex and has not been reported with oculomotor nerve palsies. This report expands the rare presentations of AL-amyloidosis.

Authors/Disclosures
Jane Liao, MD (St. Michael's Hospital) PRESENTER	No disclosure on file
Farah El-Sadi, MD	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Charles Kassardjian, MD, F�鶹��ýӳ�� (St. Michael's Hospital - Clinical Neurophysiology)	Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi Genzyme. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Astra Zeneca. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson and Johnson. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenx. Dr. Kassardjian has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi Genzyme.

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