0071643192.pdf

DIFFERENTIAL

■ Spinal cord tumors, Guillain-Barre ́ syndrome

DIAGNOSIS/TREATMENT

■ MRI to exclude compressive lesion

■ Steroids (uncertain benefit)

■ Only 50% have fair to good recovery.

Syringomyelia

Syringomyelia results from a CSF fluid collection (syrinx) within the spinal

cord. Any location is possible, but it is most commonly seen in the cervical

spine in association with Arnold-Chiari malformation.

SYMPTOMS/EXAM

■ Depend on location of syrinx

■ Pain

■ Disassociative anesthesia: Loss of pain and temperature sensation with

preservation of proprioception and light touch

■ Weakness, spasticity

DIAGNOSIS/TREATMENT

■ MRI

■ Treated with surgery, if progressive symptoms

A 55-year-old male presents with complaint of progressive weakness of

arms and legs and dry mouth. ROS is positive for significant weight loss over

the preceding months. Exam is significant for proximal muscle weakness

that improves with repeated use. This is most consistent with which disease of the

neuromuscular junction?

Lambert-Eaton myasthenic syndrome, where antibodies form to the neuro-

muscular junction. This may be a solitary disease, but has also been seen in

association with underlying malignancy.

NEUROMUSCULAR DISORDERS

The neuromuscular unit is made up of the anterior horn cells, the peripheral

nerve, the neuromuscular junction, and the muscle. Neuromuscular disorders

are a large group of disorders that result in degeneration and atrophy of mus-

cles or nerve tissue. They can be grouped based on the location of pathology

(Table 15.15).

Guillain-Barré Syndrome

GBS is primarily an acute demyelinating disorder of the peripheral nerve.

Variants include early cranial nerve findings and ataxia, primary sensory

involvement, and autonomic involvement.

NEUROLOGY

Campylobacter jejuni

gastroenteritis is a common

precursor to GBS.