Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Demyelinating polyneuropathy with preferentially-proximal involvement

M Asahina1, S Kuwabara, M Nakajima

  • 1Department of Neurology, School of Medicine, Chiba University, Japan.

Clinical Neurology and Neurosurgery
|June 24, 1998
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Type 1 interferon signature and cytotoxic T lymphocyte activation targeted against sweat ducts in inflammatory acquired idiopathic generalized anhidrosis.

Journal of the European Academy of Dermatology and Venereology : JEADV·2023
Same author

Degranulation and shrinkage of dark cells in eccrine glands and elevated serum carcinoembryonic antigen in patients with acquired idiopathic generalized anhidrosis.

Journal of the European Academy of Dermatology and Venereology : JEADV·2017
Same author

Developmental expression of Ultraspiracle proteins in the epidermis of the tobacco hornworm, Manduca sexta, during larval life and the onset of metamorphosis.

Development genes and evolution·2016
Same author

Preserved autonomic function in patients with POEMS syndrome.

Journal of the neurological sciences·2012
Same author

Reduced perfusion in the anterior cingulate cortex of patients with pure autonomic failure: an 123I-IMP SPECT study.

Journal of neurology, neurosurgery, and psychiatry·2009
Same author

Sudomotor, skin vasomotor, and cardiovascular reflexes in 3 clinical forms of Lewy body disease.

Neurology·2009

This study presents a case of limb weakness mimicking motor neuron disease, later identified as a rare form of chronic inflammatory demyelinating polyneuropathy (CIDP) with proximal demyelination. Treatment with prednisolone led to significant improvement, highlighting CIDP

Area of Science:

  • Neurology
  • Clinical Electrophysiology

Background:

  • Motor neuron disease (MND) presents with progressive limb weakness and muscle atrophy.
  • Distinguishing MND from other neuropathies is crucial for appropriate treatment.

Observation:

  • A 47-year-old man experienced 6 months of progressive, symmetrical limb weakness, muscle atrophy, and fasciculations.
  • Initial electrophysiological tests showed acute denervation without abnormalities in distal motor conduction.
  • Proximal stimulation revealed reduced compound muscle action potential (CMAP) amplitudes in median and ulnar nerves.

Findings:

  • The patient's symptoms and electrophysiological findings improved after prednisolone administration.
  • This suggests a demyelinative process, specifically chronic inflammatory demyelinating polyneuropathy (CIDP).

Related Experiment Videos

  • Demyelinative abnormalities were localized to proximal nerve segments, mimicking MND initially.

    • Implications:

    • CIDP can present with proximal demyelination, challenging initial diagnosis.
    • Early recognition and treatment of this CIDP variant can lead to significant recovery.
    • Electrophysiological assessment, including proximal stimulation, is vital for diagnosing atypical neuropathies.