RT Journal Article
SR Electronic
T1 Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 1252
OP 1258
DO 10.1212/01.WNL.0000058901.75728.4E
VO 60
IS 8
A1 Ince, P.G.
A1 Evans, J.
A1 Knopp, M.
A1 Forster, G.
A1 Hamdalla, H.H.M.
A1 Wharton, S.B.
A1 Shaw, P.J.
YR 2003
UL http://n.neurology.org/content/60/8/1252.abstract
AB 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.