A 78-year-old woman comes to the office due to worsening clumsiness and weakness of her hands for the past several months. The patient has had difficulty performing daily activities such as buttoning shirts or tightly holding garden tools. She also reports stiffness in her legs and neck. Medical history is significant for hypertension and osteoarthritis. Physical examination shows bony outgrowth at distal and proximal interphalangeal joints. There is wasting of the intrinsic hand muscles, and grip strength is decreased bilaterally. Neck flexion elicits an electric shock–like sensation down the patient's back. Ankle reflexes are 3+ bilaterally. Which of the following is the most likely diagnosis?
Show Explanatory Sources
This elderly patient has weakness in her upper and lower extremities accompanied by atrophy in her hands and stiffness with hyperreflexia in her legs. In combination with Lhermitte sign (electric shock–like pain with neck flexion), these symptoms are most consistent with cervical spinal cord compression.
Cervical myelopathy often has an insidious onset that results from degenerative changes in the vertebral bodies, discs, and joints that causes the following:
Compression of the spinal cord, specifically the spinal cord descending tracts, leads to myelopathic symptoms with upper motor neuron (UMN) signs (eg, hyperreflexia, spastic gait, stiffness/weakness) below the lesion.
Compression of the cervical spinal nerve roots at the same level leads to radiculopathy with lower motor neuron (LMN) findings (eg, atrophy, hyporeflexia) and pain that follow a radicular pattern in the upper extremities.
Because of these combined symptoms, cervical myelopathy is sometimes referred to as a radiculomyelopathy. Sensory deficits are also common, and urinary and/or rectal sphincter dysfunction may be seen. Lhermitte sign occurs when neck flexion compresses and activates the ascending spinothalamic pain tracts but is nonspecific and seen in multiple CNS diseases (eg, multiple sclerosis, transverse myelitis).
(Choice A) Amyotrophic lateral sclerosis presents with weakness accompanied by both UMN and LMN signs. However, because it results from neurodegeneration rather than compression, neck pain is uncommon and Lhermitte sign is not typically present.
(Choice B) Inflammatory myopathies (eg, polymyositis) cause weakness and often present with gait disturbances. However, because these are disorders of the musculature, UMN signs (eg, hyperreflexia) would be unexpected.
(Choice C) Multiple sclerosis (MS) can cause a variety of neurologic deficits and is the most common reason for Lhermitte sign. However, symptoms are due to lesions in the CNS; therefore, atrophy (a LMN sign) is typically absent. In addition, MS typically occurs in women age <55 and causes relapsing and remitting symptoms.
(Choice E) Although ulnar neuropathy results in intrinsic hand weakness, which patients often describe as clumsiness, it does not generally present bilaterally. In addition, it does not explain the other symptoms (eg, Lhermitte sign, UMN signs) in this patient.
Educational objective:
Cervical myelopathy often causes both spinal cord and spinal nerve root compression, resulting in myelopathic symptoms (eg, upper motor neuron signs below the lesion) and radicular symptoms (eg, lower motor neuron signs, pain in a dermatomal/myotomal pattern). Lhermitte sign (electric shock–like pain with neck flexion) may occur.