CME

Patient Management
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Seminars in Neurology
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Copyright * 2011,
American Academy of The following Patient Management Problem was chosen to reinforce the
Neurology. All rights subject matter presented in the issue. It emphasizes decisions facing the
reserved.
practicing physician. As you read through the case you will be asked to
complete 12 questions regarding history, examination, diagnostic evalua-
tion, therapy, and management. For each item, select the single best
response.
In order to obtain CME credits, subscribers must complete this Patient
Management Problem online at www.aan.com/continuum/cme. A tally
sheet is provided with this issue to allow the option of marking answers
before entering them online. A faxable scorecard is available only upon
request to subscribers who do not have computer access or to non-
subscribers who have purchased single back issues (send an email to
ContinuumCME@aan.com).
Upon completion of the Patient Management Problem, participants may
earn up to 2 hours of AMA PRA Category 1 Creditsi. Participants have up to
3 years from the date of publication to earn CME credits. No CME will be
awarded for this issue after August 31, 2014.

Learning Objective
b Upon completion of this activity, the participant will be able to:
& Describe an approach to the evaluation of polyradiculopathies and apply
this approach in the management of a specific type of polyradiculopathy

Case
A 65-year-old man presents with a 6-month history of tingling in
his feet, burning discomfort in his thighs, slowly progressive leg
weakness, and imbalance. He has no upper extremity symptoms other
than occasional intermittent tingling in his hands. He denies diplopia,
facial numbness, or difficulty with speech or swallowing. His past
medical history is remarkable for a nasopharyngeal carcinoma that was
diagnosed 10 years ago and treated with radiation and chemotherapy.
He did not experience symptoms similar to his current concerns
during the period of cancer treatment, and he has had no recurrence of
disease. He has no weight loss, skin rash, fevers, or other constitutional
symptoms.

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b 1. Which of the following is most likely to provide useful information
regarding possible causes of this patient’s symptoms?
A. history of diabetes
B. history of migraine
C. prior cervical spine surgery
D. prior episode of optic neuritis
E. use of anticoagulant medication

The patient has no known history of other medical conditions and has had
no previous spine surgery. He is not aware of any family members with
neurologic disorders or similar symptoms. His only medication is ibuprofen
for pain and a daily multivitamin.
A complete neurologic examination is performed. The abnormal
findings on his neurologic examination include moderately severe (3/5)
bilateral symmetric weakness in his iliopsoas, hip abductors, hamstrings,
anterior tibialis, and toe extensor muscles and mild weakness in his
deltoids. Reflexes are absent in his lower extremities and moderately
reduced in his upper extremities. He has distal sensory loss to pinprick,
vibration, and joint position to his midshins bilaterally.

b 2. Which of the following is the most likely localization of this patient’s

clinical findings?
A. cervical spinal cord
B. multiple mononeuropathies
C. muscle
D. neuromuscular junction
E. polyradiculopathy

The neurologic examination findings raise the possibility of a disorder

involving the peripheral nervous system. Given the distribution of these
findings, several possible sites of dysfunction of the peripheral nervous
system may be considered.

b 3. Which of the following would be the most appropriate initial test to assist
in the localization of this patient’s underlying process?
A. acetylcholine receptor antibodies
B. MRI of the cervical spine
C. nerve conduction studies and EMG
D. serum creatine kinase
E. somatosensory-evoked potentials

Nerve conduction studies and EMG are performed and demonstrate

findings consistent with a polyradiculopathy without evidence for a
myopathy, neuromuscular junction disorder, or motor neuron disease.

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 Patient Management Problem

b 4. Which of the following electrodiagnostic features would be most useful in

narrowing the list of possible etiologies of this patient’s polyradiculopathy?
A. features of primarily axonal loss
B. features of primary demyelination
C. involvement of cervical, thoracic, and lumbar roots
D. rate of firing of fibrillation potentials
E. sensory abnormalities on nerve conduction studies

The nerve
conduction studies
and EMG findings
are compatible with
a diffuse
polyradiculopathy
most severely
involving the lumbar
and thoracic roots
with some
involvement of the
cervical roots. The
nerve conduction
studies demonstrate
normal amplitudes
with slowed
conduction
velocities (in the
mid-20s m/s) and
increased temporal
dispersion and
partial conduction
blocks in several
nerves, indicating a
primarily
demyelinating PMP FIGURE 1
process (PMP
Figure 1).

b 5. Which of the following laboratory studies would be most useful to

assess for a more commonly identified etiology of a demyelinating
polyradiculopathy?
A.hemoglobin A1C
B. Lyme serology
C. paraneoplastic antibodies
D. serum immunoelectrophoresis
E. vitamin B12

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 Several laboratory studies are performed to assess for possible etiologies
of his syndrome. His serum protein immunoelectrophoresis is normal.
Other studies, including hemoglobin A1C, sedimentation rate, antinuclear
antibody, Lyme serology, and HIV serology are also normal.

b 6. Which of the following would be the most appropriate next step in the
evaluation of this patient?
A. bone marrow biopsy
B. CSF examination
C. MRI of the brain with gadolinium
D. MRI of the lumbar spine with gadolinium
E. sural nerve biopsy

The CSF examination demonstrates two white blood cells and a protein
of 210 2g/dL. The CSF glucose level is normal, and cytology is negative for
malignant cells. Bacterial and fungal cultures and Lyme PCR are also
normal. An MRI of the lumbar spine is also performed and demonstrates
mild facet arthropathy with mild foraminal narrowing at the L5-S1
level bilaterally.

b 7. Which of the following conditions is the most likely diagnosis?

A.acute inflammatory demyelinating polyradiculopathy (AIDP)
B. Charcot-Marie-Tooth disease type 1A
C.chronic inflammatory demyelinating polyradiculopathy (CIDP)
D.monoclonal gammopathy of undetermined significance (MGUS) neuropathy
E. multiple sclerosis

b 8. Which of the following would be the best initial treatment approach for
this patient?
A. initiate azathioprine
B. initiate gabapentin
C. initiate IV immunoglobulin
D. initiate riluzole
E. observe for spontaneous remission over the next 3 months

b 9. Which of the following treatments should also be discussed with the

patient as having been proven to be an effective treatment option for his
condition?
A. cisplatinum
B. hydroxychloroquine
C. intrathecal methotrexate
D. plasmapheresis
E. pyridostigmine

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 Patient Management Problem

The patient elects to initiate IV immunoglobulin (IVIg) and demonstrates

gradual improvement in weakness and numbness and reduction in
neuropathic pain over the course of 3 months. He is maintained on
0.4 g/kg of IVIg every 2 weeks with sustained benefit for 1 year. However,
after 1 year, he begins to develop increased weakness despite an increase
in the frequency of his 0.4 g/kg of IVIg to once weekly. His examination
demonstrates increased bilateral footdrop with moderately severe
proximal leg and arm weakness and areflexia.

b 10. What would be the most appropriate next step in the management of
this patient?
A. discontinue IVIg and initiate prednisone
B. increase the IVIg frequency to 2 times a week
C. perform a sural nerve biopsy to assess for a new condition
D. prescribe bilateral ankle-foot orthoses
E. repeat CSF examination to assess for elevation of the protein level

Because the patient appears to be refractory to frequent (once weekly)

IVIg treatment, alternative disease-modifying treatments are indicated.
Prednisone is initiated at 60 mg daily, and he briefly improves. However,
after 1 month, his weakness and numbness worsen. He also develops left
hip pain, skin darkening, increased hair growth, and splenomegaly and
has a weight loss of 10 kg (22 lb). Neurologic examination demonstrates
severe distal leg and arm weakness, areflexia, and distal sensory loss as
well as moderately severe proximal weakness.

b 11. Which of the following would be the most appropriate next step in the
evaluation and management of this patient?
A. increase the prednisone dose and add azathioprine
B. obtain a chest and abdomen CT
C. perform a monoclonal protein study and metastatic bone survey
D. perform a skin biopsy and autonomic reflex screen
E. repeat the EMG and CSF studies

A repeat serum immunoelectrophoresis demonstrates a monoclonal

immunoglobulin M, and bone survey demonstrates a solitary sclerotic
lesion in his left iliac bone.

b 12. Which of the following conditions is the most likely diagnosis for this patient?
A. Guillain-Barré syndrome
B. HIV-associated polyradiculopathy
C. Lyme polyradiculopathy
D. polyneuropathy, organomegaly, endocrinopathy, monoclonal
gammopathy, and skin changes (POEMS) syndrome
E. sarcoidosis

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