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From Neuropathology (Drs. Ince and Wharton, and M. Knopp and G. Forster), Academic Unit of Pathology, and the Academic Unit of Neurology (J. Evans and Dr. Shaw), Division of Genomic Medicine, Sheffield University; and Department of Neurology (H.H.M. Hamdalla), Hull Royal Infirmary, Kingston upon Hull, UK.
Address correspondence and reprint requests to Dr. Paul G. Ince, Neuropathology, ‘E’ Floor, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK; e-mail: p.g.ince{at}shef.ac.uk
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.
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