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Table of Contents    
LETTERS TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 5  |  Page : 1485-1486

A case of narcolepsy with HLA-typing from North India


Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication17-Sep-2018

Correspondence Address:
Dr. Manjari Tripathi
Room no. 705, Department of Neurology, 7th Floor, Cardio- - Neuro Centre, AIIMS, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.241338

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How to cite this article:
Srivastava S, Ramanujam B, Ihtisham K, Tripathi M. A case of narcolepsy with HLA-typing from North India. Neurol India 2018;66:1485-6

How to cite this URL:
Srivastava S, Ramanujam B, Ihtisham K, Tripathi M. A case of narcolepsy with HLA-typing from North India. Neurol India [serial online] 2018 [cited 2018 Oct 23];66:1485-6. Available from: http://www.neurologyindia.com/text.asp?2018/66/5/1485/241338




Sir,

The term “narcolepsy” was coined by Gelineau in 1880. It is a sleep disorder with onset in young, adolescent boys in 50% of cases.[1] It is characterized by a tetrad of symptoms – excessive day-time sleepiness (EDS), cataplexy, hypnagogic and hypnopompic hallucinations, and sleep paralysis, which is caused by destruction of hypocretin-producing neurons.[2] There are only a few reports from India describing this condition, with none reporting human leucocyte antigen (HLA)-typing. The allele HLA-DQB1*06:02 was found in various populations, along with other alleles, to be associated with narcolepsy. Here, we describe the case of a young boy from the northern state of Uttar Pradesh with EDS, who was diagnosed as having narcolepsy, and his HLA-typing.

An 11-year old boy studying in the 4th grade, with normal birth and developmental history and no family history of any neurological and psychiatric illness, presented with snoring and increased body movements at night while sleeping. He had excessive sleepiness during the day (around 15–16 h), over the last 3 years. Upon waking up, he would be confused and irritable, complain of generalized weakness and headache, and would talk less with his family members. He ate much more than he did earlier, and would insist on having more food despite being completely satiated. After 4–6 months, he developed a tendency to fall suddenly while standing, which was precipitated by laughter. He stopped attending school during the episodes of hypersomnolence, and had a steady decline in his academic performance. Routine laboratory tests such as blood counts, sugars, renal, hepatic, and thyroid function tests were all within normal limits. Electroencephalography (EEG) and magnetic resonance imaging (MRI) were also found to be normal. The patient was diagnosed with narcolepsy based on the clinical criteria, and a sleep-study-polysomnography (PSG) and multiple sleep latency test (MSLT) [Figure 1]. The PSG showed an apnea hypopnea index (AHI) of 10.2, a respiratory disturbance index (RDI) of 12.6, and an average percentage saturation of oxygen (AvgSpO2) level of 96%. He had a total sleep time of 8 hours. Human leukocyte antigen (HLA) typing of this patient was done by Luminex technique (Lab Scan 100 analyzer). The HLA-DQB1*06:02 and HLA- DQB1*02:01 alleles were found to be positive. He was started on modafinil (100 mg once in the morning) and, due to a slight worsening after an initial improvement, venlafaxine was added. He has been on regular follow-up for the last 2 years and is entirely symptom-free till date.
Figure 1: Graph shows 4 Naps REM sleep stages of a patient

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Our patient had HLA-DQB1*06:02 positivity, which has been reported to be strongly associated with narcolepsy in other populations such as the Han-Chinese and Chinese.[3],[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This work was supported by Department of Biotechnology Ministrt of Science and Technology, Government of India. Project code: BT/PR3436/MED/30/651/2011.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Nevsimalova S. The diagnosis and treatment of pediatric narcolepsy. Curr Neurol Neurosci Rep 2014;14:469.  Back to cited text no. 1
    
2.
Liblau RS, Vassalli A, Seifinejad A, Tafti M. Hypocretin (orexin) biology and the pathophysiology of narcolepsy with cataplexy. Lancet Neurol 2015;14:318-28.  Back to cited text no. 2
    
3.
Chen YH, Huang YS, Chien WH, Chen CH. Association analysis of the major histocompatibility complex, class II, DQ β1 gene, HLA-DQB1, with narcolepsy in Han Chinese patients from Taiwan. Sleep Med 2013;14:1393-7.  Back to cited text no. 3
    
4.
Han F, Lin L, Li J, Dong SX, An P, Zhao L, et al. HLA-DQ association and allele competition in Chinese narcolepsy. Tissue Antigens 2012;80:328-35.  Back to cited text no. 4
    


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