PMID- 34722084
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20211102
IS  - 2168-8184 (Print)
IS  - 2168-8184 (Electronic)
IS  - 2168-8184 (Linking)
VI  - 13
IP  - 9
DP  - 2021 Sep
TI  - Chronic Immune Sensory Polyradiculoneuropathy-Plus With Diffuse Enlarged Dorsal 
      Root Ganglion and Positive Serum Human Leukocyte Antigen (HLA)-B27.
PG  - e18320
LID - 10.7759/cureus.18320 [doi]
LID - e18320
AB  - The peripheral nerve is usually spared in chronic immune sensory 
      polyradiculoneuropathy (CISP) according to a literature review; however, an 
      extended-spectrum of CISP, CISP-plus, was introduced recently. Here we report a 
      29-year-old Taiwanese man who presented with numbness and hypoesthesia in all 
      distal extremities, tightness sensation in the left posterior thigh, and sensory 
      ataxia for three months. The clinical and neurophysiological examinations 
      revealed proximal sensory abnormalities along with sural nerve involvement. The 
      elevated protein level of cerebrospinal fluid (CSF) was noted and enlarged dorsal 
      root ganglia were seen on the magnetic resonance imaging (MRI) of the whole 
      spine. Autoimmune workup showed only positive human leukocyte antigen (HLA)-B27. 
      Biopsy of the sural nerve revealed inflammatory demyelinating neuropathy. Only 
      mild improvement was noted after methylprednisolone pulse therapy (1,000mg/day) 
      for three days, and he was then treated with intravenous immunoglobulin with the 
      dosage of 2g/kg-BW followed by azathioprine, and objective improvements were 
      reported. Different from the previous case reports, CISP may also associate with 
      peripheral nerve involvements. A sural nerve biopsy could assist the diagnosis. 
      Further investigation is needed for the possible immune association between 
      CISP-plus and HLA-B27.
CI  - Copyright (c) 2021, Huang et al.
FAU - Huang, I-Chen
AU  - Huang IC
AD  - Department of Neurology, Taipei Municipal Wan Fang Hospital, Taipei Medical 
      University, Taipei, TWN.
AD  - Taipei Neuroscience Institute, Taipei Medical University, Taipei, TWN.
FAU - Chen, Wei-Yu
AU  - Chen WY
AD  - Department of Pathology, Taipei Municipal Wan Fang Hospital, Taipei Medical 
      University, Taipei, TWN.
AD  - Department of Pathology, School of Medicine, College of Medicine, Taipei Medical 
      University, Taipei, TWN.
FAU - Sung, Jia-Ying
AU  - Sung JY
AD  - Department of Neurology, Taipei Municipal Wan Fang Hospital, Taipei Medical 
      University, Taipei, TWN.
AD  - Taipei Neuroscience Institute, Taipei Medical University, Taipei, TWN.
AD  - Department of Neurology, School of Medicine, College of Medicine, Taipei Medical 
      University, Taipei, TWN.
FAU - Huang, Chih-Shan
AU  - Huang CS
AD  - Department of Neurology, Taipei Municipal Wan Fang Hospital, Taipei Medical 
      University, Taipei, TWN.
AD  - Taipei Neuroscience Institute, Taipei Medical University, Taipei, TWN.
LA  - eng
PT  - Case Reports
DEP - 20210927
PL  - United States
TA  - Cureus
JT  - Cureus
JID - 101596737
PMC - PMC8549490
OTO - NOTNLM
OT  - ataxia
OT  - azathioprine treatment
OT  - chronic immune sensory polyradiculoneuropathy
OT  - steroid treatment
OT  - sural nerve biopsy
COIS- The authors have declared that no competing interests exist.
EDAT- 2021/11/02 06:00
MHDA- 2021/11/02 06:01
PMCR- 2021/09/27
CRDT- 2021/11/01 09:21
PHST- 2021/09/27 00:00 [accepted]
PHST- 2021/11/01 09:21 [entrez]
PHST- 2021/11/02 06:00 [pubmed]
PHST- 2021/11/02 06:01 [medline]
PHST- 2021/09/27 00:00 [pmc-release]
AID - 10.7759/cureus.18320 [doi]
PST - epublish
SO  - Cureus. 2021 Sep 27;13(9):e18320. doi: 10.7759/cureus.18320. eCollection 2021 
      Sep.