DOI:10.2214/AJR.05.0573
AJR 2006; 186:S230-S232
© American Roentgen Ray Society
AJR Teaching File: Progressive Polyradiculopathy
Geoffrey P. Fletcher1 and
Catherine C. Roberts1
1 Both authors: Department of Radiology, Mayo Clinic College of Medicine, 13400
E Shea Blvd., Scottsdale, AZ 85259.
Received April 19, 2005;
accepted after revision August 31, 2005.
CONTINUING MEDICAL EDUCATION
The AJR Teaching File articles are available for .25 CME credit
for completing all three articles. They are free to ARRS members and may be
purchased by nonmembers for $10.00 each. Detailed information including
objectives, disclosure information, and how to obtain CME credit can be found
at
www.arrs.org
by selecting AJR Integrative Imaging.
Address correspondence to C. C. Roberts
(roberts.catherine{at}mayo.edu).
Keywords: brain MRI • nerves • neuroradiology • polyradiculoneuropathy • spine
Clinical History
A 41-year-old man initially presented with intermittent burning feet 5
years earlier. He now has leg weakness, decreased hand dexterity, and numbness
in the umbilical and perineal regions. Findings from a dermatologic
examination are normal.
Radiologic Description
An axial T1-weighted gadolinium-enhanced MR image through the brain
(Fig. 1A) shows hypertrophy and
enhancement of cranial nerves V, VII, and VIII. There is diffuse hypertrophy
and enhancement of all the nerve roots in the cervical (Figs.
1B and
1C) and lumbar
(Fig. 1D) spine. A sagittal
T1-weighted gadolinium-enhanced MR image through the midline cervical spine
shows no abnormal cord or meningeal enhancement
(Fig. 1E). The optic nerves
were normal.
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Fig. 1A —41-year-old man with leg weakness, decreased dexterity in hands, and
numbness in umbilical and perineal regions. Axial T1-weighted
gadolinium-enhanced MR image through internal auditory canals shows
enhancement of enlarged bilateral cranial nerves VII and VIII (large
arrows) within internal auditory canals. Geniculate ganglia and
horizontal portions of cranial nerve VII (small arrows) are also
enlarged. There is marked hypertrophy of cranial nerve V (arrowheads)
in region of Meckel's cave and extending out through foramen rotundum
(V2).
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Fig. 1B —41-year-old man with leg weakness, decreased dexterity in hands, and
numbness in umbilical and perineal regions. Sagittal T1-weighted cervical
spine MR images before (B) and after (C) gadolinium
administration show enlarged, enhancing nerve roots (arrows,
C) at all levels.
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Fig. 1C —41-year-old man with leg weakness, decreased dexterity in hands, and
numbness in umbilical and perineal regions. Sagittal T1-weighted cervical
spine MR images before (B) and after (C) gadolinium
administration show enlarged, enhancing nerve roots (arrows,
C) at all levels.
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Fig. 1D —41-year-old man with leg weakness, decreased dexterity in hands, and
numbness in umbilical and perineal regions. Sagittal T1-weighted
gadolinium-enhanced MR image through lumbar spine shows enlarged enhancing
nerve roots (arrows) at all levels.
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Fig. 1E —41-year-old man with leg weakness, decreased dexterity in hands, and
numbness in umbilical and perineal regions. Sagittal T1-weighted
gadolinium-enhanced MR image through midline cervical spine reveals no
abnormal cord or meningeal enhancement.
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Diagnosis
With the given clinical history and imaging findings, chronic inflammatory
demyelinating polyradiculoneuropathy (CIDP) is the best diagnosis.
Commentary
CIDP is a relapsing or chronic progressive polyradiculopathy. It is one of
the hypertrophic polyneuropathies, which also include Charcot-Marie-Tooth
disease [1]. Demyelination in
CIDP is immune-mediated. Classification of the hypertrophic neuropathies has
been undergoing change due to advances in molecular genetics
[2].
Radiographically, hypertrophic neuropathies may be easily confused with
neurofibromatosis 2 (NF2) because of nerve root and cranial nerve enlargement
and enhancement. Spinal root and plexus hypertrophy occurs in some patients
[3], usually those with a long
duration of disease. Rarely, patients may have symptoms of a cauda equina
syndrome or a myelopathy due to hypertrophy of nerve roots. Cranial nerve
involvement shown by MRI may not be apparent clinically. The clinical findings
in patients with CIDP slowly progress from peripheral sensory disturbances to
weakness and atrophy.
Nerve biopsy classically shows primary demyelination and axonal
degeneration, which may be accompanied by inflammatory cells and
"onion-bulb formation." The onion-bulb formation is due to
multiple episodes of demyelination and remyelination
[4]. CSF examination shows
markedly increased protein [1].
Nerve conduction studies typically show multifocal conduction slowing, focal
block, and temporal dispersion. Patients may benefit from treatment with
corticosteroids, plasma exchange, and IV immune globulin
[5].
Objective
The educational objective of this article is to teach the typical
appearance of CIDP.
Conclusion
CIDP is a chronic progressive polyradiculoneuropathy. On MRI, the cranial
nerves and nerve roots can be enhancing and prominently enlarged. In some
cases, involvement of cranial nerves VII and VIII can simulate bilateral
acoustic neuromas seen in NF2. Enlargement of the spinal nerve roots can
simulate multiple neurofibromas seen in NF1. Patients with CIDP tend to have a
slow, progressive clinical course that is often misdiagnosed initially.
References
- Mizuno K, Nagamatsu M, Hattori N, et al. Chronic inflammatory
demyelinating polyradiculoneuropathy with diffuse and massive peripheral nerve
hypertrophy: distinctive clinical and magnetic resonance imaging features.
Muscle Nerve 1998;21
: 805-808[CrossRef][Medline]
- Pareyson D. Differential diagnosis of Charcot-Marie-Tooth disease
and related neuropathies. Neurol Sci2004; 25:72
-82[Medline]
- Castillo M, Mukherji SK. MRI of enlarged dorsal ganglia, lumbar
nerve roots, and cranial nerves in polyradiculoneuropathies.
Neuroradiology 1996;38
: 516-520[Medline]
- Duggins AJ, McLeod JG, Pollard JD, et al. Spinal root and plexus
hypertrophy in chronic inflammatory demyelinating polyneuropathy.
Brain 1999; 122(Pt
7): 1383-1390[Abstract/Free Full Text]
- Czaplinski A, Steck AJ. Immune mediated neuropathies: an update on
therapeutic strategies. J Neurol 2004;251
: 127-137[Medline]
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Clinical History
Radiologic Description
