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Different Presentations of Myelopathy—A Case Series

US Neurology, 2016;12(1):22–6 DOI:


Myelopathy is a common neurologic finding and presentations may vary widely. Depending on the etiology underlying the myelopathy, treatment may be surgical, medical, or supportive. In this paper, we present three cases of myelopathy due to three different etiologies and pathogeneses. All three cases initially presented with gait disturbance, and the ensuing workup revealed rare diagnoses in two out of the three cases. These three diagnoses differ in etiology and highlight the need for a close and thorough examination and workup to arrive at the correct diagnosis, which in turn leads to appropriate treatment.
Compliance with Ethics: Informed consent was obtained from all patients for being included in the case study.
Keywords: Myelopathy, ossification of the posterior longitudinal ligament, adrenomyeloneuropathy, copper deficiency myelopathy, gait disturbance
Disclosure: Krishna Pokala, David Girard, and Said R Beydoun have nothing to declare in relation to this article. No funding was received for the publication of this article.
Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, adaptation, and reproduction provided the original author(s) and source are given appropriate credit.
Received: January 21, 2016 Accepted: February 09, 2016
Correspondence: Krishna Pokala, University of Southern California, Department of Neurology, 1520 San Pablo St, Suite 3000, Los Angeles, CA 90033, US E:

Myelopathy is a common diagnosis often made by clinical neurologists and can be a cause of significant disability. Differential diagnosis classically includes structural etiology, demyelinating, metabolic, nutritional, inherited, and degenerative causes, resulting in a vast array of pathophysiologic processes, underlying myelopathy (Table 1). It is critical that clinicians use their clinical knowledge and utilize appropriate diagnostic studies in order to arrive at the correct diagnosis. This will ensure appropriate and timely management and counseling. Here, we present and discuss three unusual cases presenting with myelopathy, within a three-month period, and their respective workup and uncommon diagnoses. All patients were seen at the Neuromuscular Division of the Department of Neurology at the University of Southern California Keck Medical Center, US. Each of these cases reveals an unexpected diagnosis and highlights the importance of a broad differential diagnosis when confronted with a patient who may exhibit signs of myelopathy.

Case Presentations
Case One
A 44-year-old female physician with a history of hyperthyroidism was referred to our neuromuscular division for evaluation of abnormal gait, right leg weakness and cramping of five weeks’ duration. The patient had recently vacationed in Africa, when she noted the occurrence of pain in her right thigh, followed by a noticeable limp in her gait. Over the next few weeks, she noted worsening in her gait, the inability to run or walk long distances, and the occurrence of cramping pain in her legs. She denied back or neck pain, radicular pain, bowel or bladder symptoms, cranial nerve related complaints, or other neurologic deficit. As a physician, she became extremely concerned and anxious that she might be developing a serious, progressive, degenerative neurological ailment such as multiple sclerosis or amyotrophic lateral sclerosis.

Physical Examination
Pertinent findings on examination revealed increased muscle tone, predominantly in the right lower extremity, mild right hip flexion, knee flexion, and ankle dorsiflexion weakness. There was slight decrease in joint position and vibration sensation at the toes bilaterally. She exhibited a spastic gait on the right with inability to heel walk or tandem walk. She had difficulty squatting. She was diffusely hyper-reflexic with sustained right ankle clonus, positive crossed adductor, suprapatellar, and pectoralis reflexes, bilateral plantar extensor responses, and positive Hoffmann’s signs.

Diagnostic Studies
Initial workup including thyroid-stimulating hormone (TSH) blood test, vitamin B12 level, methylmalonic acid, homocysteine, serum copper, and human T-lymphotropic virus 1 (HTLV-1) antibody were all normal. Magnetic resonance imaging (MRI) of the brain, cervical and thoracic spine, with and without contrast, was performed.

A previous MRI, which was done at an outside facility, of her lumbar spine revealed moderate canal stenosis at L2-S1 with some ossification of the

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US Neurology - Volume 12 Issue 1 - Spring 2016
Keywords: Myelopathy, ossification of the posterior longitudinal ligament, adrenomyeloneuropathy, copper deficiency myelopathy, gait disturbance