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Idiopathic Multicentric Castleman Disease Presenting as Recurrent Acute-Onset Demyelinating Neuropathy
Chisato Saito1, Yumi Honda1, Hironori Mizutani1
1Department of Neurology, Kumamoto University Hospital, Japan.
This case study describes a rare instance of acute, severe motor-predominant polyneuropathy in a patient diagnosed with idiopathic multicentric Castleman disease (iMCD). The findings highlight the potential role of interleukin-6 (IL-6) in iMCD-associated neuropathy.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Idiopathic multicentric Castleman disease (iMCD) typically presents with gradual sensory neuropathy.
- Acute, severe, motor-predominant polyneuropathy is an exceptionally rare manifestation of iMCD.
Purpose of the Study:
- To report an unusual case of iMCD presenting with acute motor-predominant polyneuropathy.
- To explore the potential role of interleukin-6 (IL-6) in the pathogenesis of this neuropathy.
Main Methods:
- Clinical presentation and neurological examination.
- Nerve conduction studies and cerebrospinal fluid analysis.
- Serological testing for anti-GM1/GM2 IgM antibodies, IL-6 levels, FDG-PET imaging, and lymph node biopsy.
Main Results:
- The patient exhibited recurrent, motor-predominant polyneuropathy with fever and arthralgia, leading to loss of ambulation.
- Nerve conduction studies showed demyelination, and CSF analysis revealed albuminocytological dissociation.
- Elevated IL-6 levels, diffuse lymphadenopathy, and confirmed iMCD diagnosis were observed.
Conclusions:
- This case underscores that iMCD can present with acute, severe, motor-predominant neuropathy, challenging typical presentations.
- Markedly elevated IL-6 levels suggest a significant role in the IL-6-mediated neuropathy observed in this iMCD patient.
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