Spinal cord compression from hypertrophic nerve roots in chronic inflammatory demyelinating polyradiculoneuropathy - A case report.
Charcot-marie-tooth disease
Chronic inflammatory demyelinating polyradiculoneuropathy
Hypertrophic neuropathy
Neurofibromatosis
Journal
Surgical neurology international
ISSN: 2229-5097
Titre abrégé: Surg Neurol Int
Pays: United States
ID NLM: 101535836
Informations de publication
Date de publication:
2021
2021
Historique:
received:
14
01
2021
accepted:
17
02
2021
entrez:
21
4
2021
pubmed:
22
4
2021
medline:
22
4
2021
Statut:
epublish
Résumé
Spinal cord compression secondary to nerve root hypertrophy is often attributed to hereditary neuropathies. However, to avoid misdiagnosis, rare immune-mediated neuropathy such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) should not be overlooked. This report presents a case of multilevel nerve root hypertrophy leading to significant cord compression from CIDP. We report a 56-year-old gentleman with type two diabetes mellitus who presented with subacute cervical cord syndrome following a fall. Mixed upper and lower motor neuron features were noted on examination. Magnetic resonance imaging showed significant pan-spinal proximal nerve root hypertrophy, compressing the cervical spinal cord. Initial radiological opinion raised the possibility of neurofibromatosis type 1 (NF-1), but neurophysiology revealed both axonal and demyelinating changes that were etiologically non-specific. C6 root and sural nerve biopsies taken at cervical decompression displayed striking features suggestive for CIDP. Although NF-1 is the most observed condition associated with root hypertrophy, other important and potentially treatable differentials need to be entertained. While rare, CIDP can cause significant spinal cord compression. Furthermore, clinical manifestations of CIDP can mimic those of inherited peripheral neuropathies. Neurologists and neurosurgeons should be aware of this condition to optimize subsequent therapeutic decision-making.
Sections du résumé
BACKGROUND
BACKGROUND
Spinal cord compression secondary to nerve root hypertrophy is often attributed to hereditary neuropathies. However, to avoid misdiagnosis, rare immune-mediated neuropathy such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) should not be overlooked. This report presents a case of multilevel nerve root hypertrophy leading to significant cord compression from CIDP.
CASE DESCRIPTION
METHODS
We report a 56-year-old gentleman with type two diabetes mellitus who presented with subacute cervical cord syndrome following a fall. Mixed upper and lower motor neuron features were noted on examination. Magnetic resonance imaging showed significant pan-spinal proximal nerve root hypertrophy, compressing the cervical spinal cord. Initial radiological opinion raised the possibility of neurofibromatosis type 1 (NF-1), but neurophysiology revealed both axonal and demyelinating changes that were etiologically non-specific. C6 root and sural nerve biopsies taken at cervical decompression displayed striking features suggestive for CIDP. Although NF-1 is the most observed condition associated with root hypertrophy, other important and potentially treatable differentials need to be entertained.
CONCLUSION
CONCLUSIONS
While rare, CIDP can cause significant spinal cord compression. Furthermore, clinical manifestations of CIDP can mimic those of inherited peripheral neuropathies. Neurologists and neurosurgeons should be aware of this condition to optimize subsequent therapeutic decision-making.
Identifiants
pubmed: 33880219
doi: 10.25259/SNI_35_2021
pii: 10.25259/SNI_35_2021
pmc: PMC8053436
doi:
Types de publication
Case Reports
Langues
eng
Pagination
114Informations de copyright
Copyright: © 2021 Surgical Neurology International.
Déclaration de conflit d'intérêts
There are no conflicts of interest.
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