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Mitochondrial proliferation leading to ragged appearance in Gomori trichrome stain
10%
2/21
Perimysial CD4+ infiltration and perifascicular atrophy
33%
7/21
CD8+ infiltrating within the fascicle
19%
4/21
Denervation and reinnervation of the muscle
29%
6/21
Larval cysts
0%
0/21
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This patient presents with both upper and lower motor neuron signs, and intact sensation. This, along with tongue fasciculations, and trouble swallowing is suggestive of Amyotrophic Lateral Sclerosis (ALS). Denervation and reinnervation will be seen on muscle biopsy. ALS is a neurodegenerative disorder that affects the motor neurons of the central nervous system. This is mainly a sporadic disorder (90%); however, mutations in superoxide dismutase 1 (SOD-1) is associated with certain familial forms of this condition. Given that SOD-1 is involved in neutralizing reactive oxygen species, this suggests that neuronal cell death may be mediated by free radical toxicity. Etiology is unclear, but may be multifactorial. Patients present with asymmetric upper and lower motor neuron symptoms, with preservation of mentation, bowel, bladder, sensory, and sexual function. Walling presents a review of ALS. Patients with ALS classically present with asymmetric muscle weakness and atrophy, of progressive and insidious nature. In about 25% of cases, bulbar symptoms may manifest, leading to events such as drooling secondary to difficulties in swallowing liquids than with solids. Patients eventually reach a state of profound disability, requiring ventilatory assistance and gastrostomy for nutrition. Patients usually die secondary to respiratory failure. Zarei et al. further discuss riluzole, an FDA-approved drug that has been identified to have a beneficial effect on survival in patients with ALS. The mechanism of action of this medication is of debate. However, it has been recognized that it decreases glutamate’s neurotoxic effects, has NMDA receptor antagonistic effects, and inactivates voltage-dependent sodium channels. Incorrect Answers: Answer 1: This describes mitochondrial myopathies, such as Myoclonic Epilepsy with Ragged Red Fibers (MERRF syndrome). Answer 2: This is suggestive of dermatomyositis. These patients can present with symmetric proximal muscle weakness, in addition to a dermatologic findings such as “shawl and face,” gottron papules, and so forth. Answer 3: This is suggestive of polymyositis. These patients also present with symmetrical proximal muscle weakness. Answer 5: This is suggestive of a Trichinella spiralis infection, leading to cysts in striated muscle.
5.0
(7)
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