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Isolated dysphagia due to aquaporin-4 autoimmunity

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To the Editor,

Neuromyelitis optica (NMO) is an inflammatory, demyelinating disease that affects the spinal cord and optic nerves and is associated with aquaporin-4 antibodies (Aqp-aquaporin-4 Ab) (1,2). NMO patients might occasionally present with medulla symptoms such as hiccups, nausea and vomiting and with small lesions in the dorsal medulla, which corresponds to a site of high Aqp-4 expression (2,3).

A 42-year-old female was referred to us with a three-week history of intractable hiccups, nausea and vomiting. She had undergone an attack of op-tic neuritis and transverse myelitis one year ago. Her physical and neurological examinations, ro-utine blood tests, upper gastrointestinal

endos-copy, and cranial and spinal magnetic resonance imaging (MRI) with gadolinium enhancement we-re normal. Ten days later, she developed a sudden respiratory arrest, was intubated and her symp-toms resolved spontaneously in one week. Thirty-six days later, she admitted with a two-day history of dysphagia. Her uvula was in the midline, the soft palate did not elevate on phonation, and the gag reflex could not be elicited. The neurological examination was otherwise normal. The T2-we-ighted cranial MRI sections revealed a large hype-rintense lesion in the medulla oblongata (Figure 1). The cerebrospinal fluid examination showed lymphocytosis (24/mm3), increased protein (54

Isolated dysphagia due to aquaporin-4 autoimmunity

Aquaporin-4 otoimmünitesine ba¤l› izole disfaji

Manuscript received:18.08.2011Accepted:28.11.2011

Turk J Gastroenterol 2012; 23 (6): 804-831 doi:10.4318/tjg.2012.0437

Address for correspondence:Erdem TÜZÜN Department of Neurology, ‹stanbul University

Istanbul Faculty of Medicine, 34390 Çapa, ‹stanbul, Turkey Phone: + 90 212 414 20 00/ ext: 32580 • Fax: + 90 212 533 43 93 E-mail: drerdem@yahoo.com

F

Fiigguurree 11.. The T2-weighted axial magnetic resonance images show an area of hyperintensity in the medulla oblongata.

LETTERS TO THE EDITOR

(2)

mg/dl) and normal glucose concentration and oli-goclonal bands. Visual evoked potentials and a comprehensive screening for infectious and syste-mic autoimmune disorders were normal. Intrave-nous (i.v.) 1000 mg methylprednisolone treatment was administered for five days, and her dysphagi-a resolved in two weeks. In the following 14 yedysphagi-ars, she developed three transverse myelitis attacks. Aqp-4 Ab was detected in the archived sera obtai-ned during the brainstem and myelitis attacks using a cell-based assay with Aqp-4-transfected HEK-293 cells (1).

Isolated dysphagia is a rare type of presentation for most neurological diseases. Dysphagia has been re-ported in a few NMO patients in association with

other symptoms (4,5). Hiccups, nausea and vomi-ting presumably occur due to the involvement of the area postrema, located in the dorsal medulla. The solitary tract and dorsal vagal nuclei are loca-ted in close proximity to the area postrema. Howe-ver, involvement of these nuclei alone apparently does not cause dysphagia (2). Dysphagia is expec-ted to occur due to the involvement of the nucleus ambiguus, which is far more ventrally located and is thus spared in most cases. Our patient’s findings show that medulla lesions due to NMO are not ne-cessarily confined to the dorsal medulla and might extend ventrally, causing dysphagia. NMO should thus be suspected in patients presenting with dysphagia or other ventral medulla symptoms.

Letters to the editor

805

Manuscript received:21.06.2011Accepted:10.07.2011

doi:10.4318/tjg.2012.0405

Address for correspondence:Altay ÇELEB‹

Kocaeli University School of Medicine, Department of Gastroenterology, Kocaeli, Turkey

Phone: + 90 262 303 75 29 E-mail: altaycelebi@yahoo.com REFERENCES

1. Waters P, Jarius S, Littleton E, et al. Aquaporin-4 antibo-dies in neuromyelitis optica and longitudinally extensive transverse myelitis. Arch Neurol 2008; 65: 913-9.

2. Apiwattanakul M, Popescu BF, Matiello M, et al. Intrac-table vomiting as the initial presentation of neuromyelitis optica. Ann Neurol 2010; 68: 757-61.

3. Riphagen J, Modderman P, Verrips A. Hiccups, nausea, and vomiting: water channels under attack! Lancet 2010; 375: 954.

4. Kobayashi Z, Tsuchiya K, Uchihara T, et al. Intractable hiccup caused by medulla oblongata lesions: a study of an autopsy patient with possible neuromyelitis optica. J Neu-rol Sci 2009; 285: 241-5.

5. Lotze TE, Northrop JL, Hutton GJ, et al. Spectrum of pedi-atric neuromyelitis optica. Pedipedi-atrics 2008; 122: e1039-47.

Recai TÜRKO⁄LU2, Asl› KIYAT-ATAMER3,

Erdem TÜZÜN1

, Gülflen AKMAN-DEM‹R3

Department of 1Neurology, ‹stanbul Faculty of Medicine,

‹stanbul

Department of 2Neurology, Haydarpasa Numune Education

and Research Hospital, ‹stanbul

Department of 3Neurology, ‹stanbul Bilim University, ‹stanbul

The prevalence of CYP2C19 mutations in Turkish

patients with dyspepsia and influence on H. pylori

eradication therapy

Dispeptik Türk hastalarda CYP2C19 mutasyonlar›n›n prevalans› ve bunun H. pylori

eradikasyon tedavisine etkisi

To the Editor,

We read with great interest the paper by Ozdil et al. published in your journal entitled “Influence of CYP2C19 functional polymorphism on Helicobac-ter pylori eradication” (1). In that paper, they

re-ported that cytochrome P450 2C19 (CYP2C19) polymorphism has an impact on H. pylori eradica-tion, and heterozygous CYP2C19 extensive meta-bolizers (hetero EMs) had statistically

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