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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 3  |  Page : 333-334

Pruritus: Is it a predictor of relapse in neuromyelitisoptica spectrum disorder?


Department of Neurology, PSG Institute of Medical Science and Research, Coimbatore, Tamil Nadu, India

Date of Submission10-Jun-2014
Date of Decision26-Jun-2014
Date of Acceptance15-Jun-2014
Date of Web Publication18-Jul-2014

Correspondence Address:
E Prasanna Venkatesan
Department of Neurology, PSG Institute of Medical Science and Research, Coimbatore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.137020

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How to cite this article:
Ramasamy B, Kalidoss R, Gnanashanmugham G, Venkatesan E P. Pruritus: Is it a predictor of relapse in neuromyelitisoptica spectrum disorder?. Neurol India 2014;62:333-4

How to cite this URL:
Ramasamy B, Kalidoss R, Gnanashanmugham G, Venkatesan E P. Pruritus: Is it a predictor of relapse in neuromyelitisoptica spectrum disorder?. Neurol India [serial online] 2014 [cited 2021 Jan 16];62:333-4. Available from: https://www.neurologyindia.com/text.asp?2014/62/3/333/137020


Sir,

A 23-year-old female presented with history of recurrent vomiting and hiccups for 1 month duration. This was followed by left optic neuritis. A month later she developed left hemisensory loss, ataxia, and vertigo. Brain and spine magnetic resonance imaging (MRI) showed demyelination in dorsal medulla extending into upper cervical cord (C4-5). AQ4 antibody was positive and she was treated with plasma exchange and later with disease modifying therapy for neuromyelitisoptica spectrum disorder (NMOSD). Patient did well for the next 6 months. She presented with pruritus confined to upper limbs and neck of 5 days duration. Pruritus was paroxysmal in the upper limbs and was very intense. She developed multiple scratch marks over the above-mentioned areas [Figure 1]a and b and also developed ulcer on shoulders. Five days after onset of pruritus she developed acute onset of weakness of left upper limb associated with paresthesias. On examination, power in left upper limb was grade 3/5 with left extensor plantar response and reduced joint position sense in left thumb. MRI cervical spine showed gadolinium enhancing hyperintense signal change from C3-7 suggestive of a longitudinally extending transverse myelitis (LETM) [Figure 2]. She was treated for the relapse and her weakness improved.
Figure 1: (a) Excoriation of skin secondary to pruritus (b) Excoriation of skin and multiple scratch marks secondary to pruritus

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Figure 2: MRI showing LETM from C3 to C7

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Pruritus is defined as an unpleasant sensation that provokes the desire to scratch. Pruritus is commonly seen in systemic diseases like renal failure, diabetes, liver disease, malignancy, and idiopathic pruritus. It is rather an uncommon manifestation of neurological diseases. The sensation of pruritus is transmitted through slow-conducting unmyelinated C fiber located near the dermoepidermal junction. These neurons are activated by histamine, substance P, serotonin, bradykinin, proteases, and endothelin. [1]

Paroxysmal intense pruritus can occur in both multiple sclerosis (MS) and NMO. In a study of the 377 MS patients, 18 patients had pruritus (Mattew et al. not cited in the references). Of the 45 AQ4-positive NMO patients, 12 (27.3%) patients had pruritus and the authors concluded that pruritus is under recognized and not frequently reported feature of NMO. [2] Review of literature showed only one case report of NMO who had pruritus as a first symptom of relapse. [3] The pathophysiology of paroxysmal itching is believed to be due to transversely spreading ephaptic activation of axons lying in a partially demyelinated lesion in fiber tracts in the central nervous system. We believe that when a partial demyelinating lesion that is not sufficient to produce a permanent deficit, leads to minor irritation of surrounding axons which may cause paroxysmal pruritus. [4]

In conclusion, paroxysmal pruritus is an uncommon but under recognized symptom of NMO. Systemic diseases and drug allergy must be excluded before pruritus is considered as a symptom of NMO. It can very rarely present as first symptom of relapse in NMO and precedes the development of myelopathy by several days. One should predict impending relapse of myelopathy when a patient with NMO presents with itching.

 
  References Top

1.Cho YL, Liu HN, Huang TP, Tarng DC. Uremic pruritus: Roles of parathyroid hormone and substance P. J Am Acad Dermatol 1997;36:538-43.  Back to cited text no. 1
    
2.Elsone L, Townsend T, Mutch K, Das K, Boggild M, Nurmikko T, et al. Neuropathic pruritus (itch) in neuromyelitisoptica. Mult Scler 2013;19:475-9.  Back to cited text no. 2
    
3.Sang-Soo L, Hyung-Suk L, Shin-Hye B. Paroxysmal pruritus as the first relapsing symptom of neuromyelitisoptica. Neurology Asia 2010;15:185-7.  Back to cited text no. 3
    
4.Osterman PO, Westerberg CE. Paroxysmal attacks in multiple sclerosis. Brain 1975;98:189-202.  Back to cited text no. 4
    


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