MR imaging in hypertrophic neuropathy: A case of hereditary motor and sensory neuropathy, type i (charcot-marie-tooth)

Choi, S. W.; Bowers, Roger P.; Buckthal, Paul E.

doi:10.1016/0899-7071(90)90073-k

Cited by 23 publications

(5 citation statements)

References 8 publications

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“…23 The usual clinical presentation is chronic degeneration of peripheral nerves and roots, with muscle atrophy and sensory impairment in a distal distribution. 15,24 Clinical findings include atrophy and sensory loss affecting all extremities, ataxia, areflexia, palpably enlarged peripheral nerves, pes cavus, and hammer toes. Bilateral lumbosacral plexus and peripheral nerve involvement are common.…”

Section: Infectious Neuropathymentioning

confidence: 99%

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High-Resolution MR Neurography of Diffuse Peripheral Nerve Lesions

Thawait¹,

Chaudhry²,

Thawait³

et al. 2010

AJNR Am J Neuroradiol

172

103

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SUMMARY:High-resolution MR imaging of peripheral nerves is becoming more common and practical with the increasing availability of 3T magnets. There are multiple reports of MR imaging of peripheral nerves in compression and entrapment neuropathies. However, there is a relative paucity of literature on MRN appearance of diffuse peripheral nerve lesions. We attempted to highlight the salient imaging features of myriad diffuse peripheral nerve disorders and imaging techniques for MRN. Using clinical and pathologically proved relevant examples, we present the MRN appearance of various types of diffuse peripheral nerve lesions, such as traumatic, inflammatory, infectious, hereditary, radiationinduced, neoplastic, and tumor variants.ABBREVIATIONS: CIDP ϭ chronic inflammatory demyelinating polyneuropathy; CMT ϭ CharcotMarie-Tooth; fat sat ϭ fat saturated; FLAIR ϭ fluid-attenuated inversion recovery; FLH ϭ fibrolipomatous hamartoma; GBS ϭ Guillain Barré syndrome; CMT/HSMN ϭ Charcot-Marie-Tooth/hereditary motor and sensory neuropathy; MMN ϭ multifocal motor neuropathy; MPNST ϭ malignant peripheral nerve sheath tumor; MRN ϭ MR neurography; NF1 ϭ neurofibromatosis type 1; NL ϭ neurolymphomatosis; SE ϭ spin-echo; SNR ϭ signal-to-noise ratio; SPACE ϭ sampling perfection with application-optimized contrasts by using different flip angle evolutions; SPAIR ϭ spectralattenuated inversion recovery; STIR ϭ short-tau inversion recovery; T1WI ϭ T1-weighted imaging; T1WIFS ϭ T1-weighted fat-saturated imaging; T2WI ϭ T2-weighted imaging; T2WIFS ϭ T2-weighted fat-saturated imaging H igh-resolution MR imaging of peripheral nerves is becoming more common and practical with increasing availability of 3T magnets. These magnets provide high SNR, which can be used for a quicker acquisition time as well as higher image contrast and resolution. There have been multiple reports of MR imaging of peripheral nerves in compression and entrapment neuropathies.1-3 However, there is a relative paucity of literature on the MRN appearance of diffuse peripheral nerve lesions. 4 These lesions seen on MR imaging present a diagnostic dilemma because a long list of pathologies could be causing them. We attempt to highlight the salient imaging features of myriad diffuse peripheral nerve disorders and to describe a diagnostic approach to these lesions on the basis of the available literature and our experience in this area. Various clinical and pathologically proved relevant examples of these pathologies are illustrated. . T1WI demonstrates fat planes delineating the normal nerve (perineural fat). C, Axial STIR SPACE at the level of the thighs shows an abnormal sciatic nerve. Notice the enlarged size and T2 hyperintensity of the fascicles. The dark rim of perineural fat is also disrupted. Also note posterior compartment denervation muscle edema (arrows).

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Section: Infectious Neuropathymentioning

confidence: 99%

“…[10][11][12][13][14][15][16][17][18] Relevant examples of cases from each category with their MRN imaging characteristics are illustrated (Table 3).…”

Section: Etiology Of Diffuse Peripheral Nerve Lesionsmentioning

confidence: 99%

High-Resolution MR Neurography of Diffuse Peripheral Nerve Lesions

Thawait¹,

Chaudhry²,

Thawait³

et al. 2010

AJNR Am J Neuroradiol

172

103

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“…Root enhancement also has been observed in 2 patients with acute inflammatory polyradiculoneuropathy (Guillain-Barre syndrome [GBS]) [9, I 0] and I patient with chronic inflammatory demyelinating polyneuropathy (CIDP) [II]. It is not clear whether the enhancement seen in these patients was caused by demyelination or was secondary to the inflammatory process, since faint enhancement also has been observed on MRis of a patient with hereditary motor sensory neuropathy (HMSN) type I [ 12].In this study, contrast-enhanced MRI was performed in patients with CIDP, GBS, or the Miller-Fisher variant of GBS, and the findings were compared with those of 8 control…”

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confidence: 99%

Contrast‐enhanced Magnetic Resonance Imaging of the Lumbosacral Roots in the Dysimmune Inflammatory Polyneuropathies

Bertorini

Halford

Lawrence

et al. 1995

Journal of Neuroimaging

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The diagnosis of acute Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy is based on clinical characteristics, abnormalities on nerve conduction studies, and nerve biopsy specimens indicating demyelination. Inflammation and edema are also common findings in nerve specimens. Immunotherapy is helpful in these dysimmune conditions. Occasionally the diagnosis is difficult to make, particularly when electrophysiological testing or nerve biopsy findings are not characteristic. The authors found contrast enhancement of lumbosacral roots in patients with chronic inflammatory demyelinating polyradiculoneuropathy and Guillain-Barré syndrome, but not in those with other demyelinating neuropathies. Contrast-enhanced magnetic resonance imaging could be a useful tool in the diagnosis of the dysimmune inflammatory neuropathies.

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“…Concomitant nerve root enlargement at the cervical and thoracic levels is not uncommon and has been described in several case reports. Post‐contrast, the enlarged nerve roots show faint, if any, enhancement 3–6 . Bilateral enlarged nerve roots are not specific for CMT and have been described with a host of other conditions, including neurofibromatosis, Chronic Inflammatory Demyelinating Polyneuropathy, Guillia‐Barre syndrome, sarcoidosis, arachnoditis, memingitis, metastatic disease, lymphomatous/leukemic infiltration, acromegaly or amyloidosis.…”

Section: Discussionmentioning

confidence: 99%

“…Classically, these disorders are characterized by progressive motor weakness, decreased nerve conduction velocities and nerve root enlargement 1,2 . The imaging literature for this disease focuses on the identification of nerve hypertrophy with faint, if any, nerve root enhancement 3–6 . We present a patient with CMT who had calcification of multiple spinal nerve roots in the cervical spine.…”

Section: Introductionmentioning

confidence: 99%

Multiple bilateral spinal nerve root calcifications in Charcot‐Marie‐Tooth disease

2009

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We present a patient with Charcot-Marie-Tooth disease with multiple bilateral symmetrical cervical nerve root calcifications. To our knowledge, such a finding has not been described in the literature in association with this disease. We propose that multiple bilateral symmetrical spinal nerve root calcifications may be an additional imaging feature, possibly diagnostic, in this hereditary motor and spinal neuropathy.

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MR imaging in hypertrophic neuropathy: A case of hereditary motor and sensory neuropathy, type i (charcot-marie-tooth)

Cited by 23 publications

References 8 publications

High-Resolution MR Neurography of Diffuse Peripheral Nerve Lesions

High-Resolution MR Neurography of Diffuse Peripheral Nerve Lesions

Contrast‐enhanced Magnetic Resonance Imaging of the Lumbosacral Roots in the Dysimmune Inflammatory Polyneuropathies

Multiple bilateral spinal nerve root calcifications in Charcot‐Marie‐Tooth disease

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