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Neurology. 2003 Apr 22;60(8):1252-8.

Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS.

Author information

1
Neuropathology, Academic Unit of Pathology, Division of Genomic Medicine, Sheffield University, UK. p.g.ince@shef.ac.uk

Abstract

OBJECTIVE:

Examining the unresolved relationship between the lower motor neuron disorder progressive muscular atrophy (PMA) and ALS is important in clinical practice because of emerging therapies.

METHODS:

Spinal and brainstem tissues donated from patients with ALS/motor neuron disorder (n = 81) were examined. Using retrospective case note review, the authors assigned patients into three categories: PMA (12), PMA progressing to ALS (6), and ALS ab initio (63). Conventional stains for long tract degeneration and immunocytochemistry for ubiquitin and the macrophage marker CD68 were examined.

RESULTS:

Rapid progression and typical ubiquitinated inclusions in lower motor neurons were present in 77 (95%) of the cases. Immunocytochemistry for CD68 was a more sensitive marker of long tract pathology in comparison with conventional stains. Half of the cases with PMA showed corticospinal tract degeneration by CD68.

CONCLUSION:

Patients with PMA frequently have undetected long tract pathology and most have ubiquitinated inclusions typical of ALS. A patient presenting with PMA with rapid clinical evolution likely has the pathology and pathophysiology of ALS whether or not upper motor neuron signs evolve.

PMID:
12707426
[Indexed for MEDLINE]

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