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| CME QUIZ |
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| Year : 2019 | Volume
: 17
| Issue : 4 | Page : 155-156 |
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Mimicking amyotrophic lateral sclerosis: Cervical spondylotic myelopathy
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
Department of Neurology, Federal University of Santa Maria, Santa Maria, Rio Grande do Sul, Brazil
| Date of Submission | 30-Aug-2019 |
| Date of Decision | 22-Sep-2019 |
| Date of Acceptance | 15-Oct-2019 |
| Date of Web Publication | 12-Dec-2019 |
Correspondence Address:
Mr. Jamir Pitton Rissardo
Av. Roraima n˚ 1000, Cidade Universitária, Bairro Camobi, Santa Maria - RS 97105-900
Brazil
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/cmi.cmi_36_19
How to cite this article:
Rissardo JP, Caprara AL. Mimicking amyotrophic lateral sclerosis: Cervical spondylotic myelopathy. Curr Med Issues 2019;17:155-6 |
| Case Scenario | |  |
An 80-year-old male was admitted to our hospital for urinary catheter change. The patient stated that he went to other clinic 1 year ago because he had experienced progressive weakness in all extremities and leaky bladder. The neurological examination at that time showed a wheelchair patient with weakness in the upper and lower limbs, fasciculations in the upper limb, left plantar extension, and hyperreflexia in the upper and lower limbs. Laboratory tests and brain magnetic resonance imaging (MRI) were normal. A diagnosis of amyotrophic lateral sclerosis (ALS) was made, and riluzole 50 mg twice daily was started. On follow-up, he reported relative improvement of urinary incontinence and deambulation.
On admission in our hospital, after the urinary catheter was changed, he also mentioned cervicalgia with >6 months of onset. His family history was unremarkable. Physical examination showed a wheelchair patient with the previous neurological signs described in addition to decreased tactile sensitivity. Laboratory tests were within normal limits. Electromyography showed increased insertional activity, sharp positive waves, fasciculations, and a diminished motor unit recruitment with prolonged central motor conduction time. In addition, sensory amplitudes and velocities in the upper limb were abnormal. A cervical MRI was requested [Figure 1]. | Figure 1: Sagittal T1-weighted (a), sagittal T2-weighted (b), and frontal spectral attenuated inversion recovery (c) views of MRI
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| Questions | | |
- What are the findings in the cervical spine MRI?
- What is the most likely diagnosis taking into account this clinical scenario?
- What is the management of this condition?
| Answers | | |
1. Neuroimages of cervical spine showing narrowing of spinal canal between C3 and C5. Sagittal T1-weighted (A), sagittal T2-weighted (B), and frontal spectral attenuated inversion recovery (C) views of MRI [Figure 2] and [Figure 3]. | Figure 2: Neuroimages of cervical spine showing narrowing of spinal canal between C3 and C5. Sagittal T1-weighted (a), sagittal T2-weighted (b), and frontal spectral attenuated inversion recovery (c) views of MRI
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 | Figure 3: T2-weighted brain magnetic resonance imaging showing mild hypersignal along the cervical spine suggestive of myelopathy.
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2. Cervical spondylotic myelopathy (CSM).
ALS is a progressive neurodegenerative disorder affecting the upper and lower motor neurons. Severe disability and death eventually occur along the disease course. The incidence rate is about 1–3 cases/100,000.[1] A diverse group of conditions can cause clinical manifestations that resemble those of ALS. CSM corresponds approximately to 3% of these mimicking syndromes.[2] In this context, the differentiation of these two entities is crucial and sometimes challenging because degenerative changes of spine frequently coexist in elderly patients with ALS. In the present report, the initial evaluation in the other clinic was based on brain MRI. However, sensory or bowel/bladder deficits do not occur commonly in ALS and when inferior limbs are involved, cervicothoracic imaging should be requested.
Neurological examination in both disorders frequently reveals upper and lower motor neuron deficits. In this context, CSM is associated with lower motor neuron signs isolated to the affected cervical myotomes, but in ALS, they commonly appear in the legs and cranial muscles.[3] Sensory deficits are not expected in ALS, but these could be abrupt or absent in patients with CSM. Therefore, electrophysiologic studies are needed to better differentiate these two entities, and in CSM, they may reveal the presence and degree of anterior horn cell or spinal nerve route damage.[4],[5]
3. Conservative measures or surgical decompression.
Management choices for CSM remain controversial, mainly when and if is necessary to operate a patient. One of these options is conservative measures that include intermittent neck immobilization, pain management, and restriction of high-risk activities. However, patients that chose this choice or who are not eligible for surgery should have a close neurologic follow-up to assess their spinal deterioration. In some cases, the acute deterioration could occur in individuals with CSM, which is a neurological emergency.[4]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Logroscino G, Traynor BJ, Hardiman O, Chiò A, Mitchell D, Swingler RJ, et al. Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 2010;81:385-90.  |
| 2. | Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman O. Amyotrophic lateral sclerosis mimic syndromes: A population-based study. Arch Neurol 2000;57:109-13. |
| 3. | Dvorak J, Sutter M, Herdmann J. Cervical myelopathy: Clinical and neurophysiological evaluation. Eur Spine J 2003;12 Suppl 2:S181-7. |
| 4. | Hardiman O, van den Berg LH, Kiernan MC. Clinical diagnosis and management of amyotrophic lateral sclerosis. Nat Rev Neurol 2011;7:639-49. |
| 5. | Chiles BW 3 rd, Leonard MA, Choudhri HF, Cooper PR. Cervical spondylotic myelopathy: Patterns of neurological deficit and recovery after anterior cervical decompression. Neurosurgery 1999;44:762-9. |
[Figure 1], [Figure 2], [Figure 3]
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