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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
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Primary amyloidosis presenting as upper limb multiple mononeuropathies.

Jennifer A Tracy1, Peter J Dyck, P James B Dyck

  • 1Peripheral Neuropathy Research Laboratory, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.

Muscle & Nerve
|April 21, 2010
PubMed
Summary
This summary is machine-generated.

Primary amyloidosis typically causes lower limb neuropathy. This case highlights it can also manifest as progressive upper limb mononeuropathies, expanding diagnostic considerations for this condition.

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Area of Science:

  • Neurology
  • Oncology
  • Pathology

Background:

  • Primary (AL) amyloidosis commonly presents with peripheral neuropathy, typically affecting lower limbs symmetrically and predominantly small nerve fibers.
  • This neuropathy is usually length-dependent, impacting sensory and autonomic functions significantly.

Observation:

  • A unique case of a patient presenting with stepwise progressive, multiple upper limb mononeuropathies is described.
  • Nerve biopsy confirmed the cause to be primary amyloidosis due to lambda light chain deposition.

Findings:

  • The patient's presentation deviated from the typical pattern, with no systemic or autonomic involvement observed.
  • This atypical presentation underscores that primary amyloidosis can manifest as a multiple mononeuropathy.

Implications:

  • The findings broaden the differential diagnosis for multiple mononeuropathy, particularly in cases without typical systemic signs.
  • Clinicians should consider light chain amyloidosis in the etiological workup of unusual mononeuropathy patterns.
  • This case emphasizes the importance of nerve biopsy for accurate diagnosis in complex neuropathic presentations.