Neuromyelitis optica presenting as intractable vomiting and hyperCKaemia

التفاصيل البيبلوغرافية
العنوان: Neuromyelitis optica presenting as intractable vomiting and hyperCKaemia
المؤلفون: Grace Harkin, Valerie Byrnes, Tarig Mohammed Abkur, John Lynch, Hugh Kearney, Eilis Foran
المصدر: Journal of Neurology. 263:171-173
بيانات النشر: Springer Science and Business Media LLC, 2015.
سنة النشر: 2015
مصطلحات موضوعية: 0301 basic medicine, medicine.medical_specialty, Neuromyelitis optica, business.industry, Myelitis, medicine.disease, Gastroenterology, Transverse myelitis, Serology, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, Cerebrospinal fluid, Neurology, Internal medicine, Vomiting, Medicine, Optic neuritis, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery, CSF albumin
الوصف: A 52-year-old woman presented with 1-week history of nausea and vomiting. Initial blood tests showed elevated creatine kinase (CK) at 1527 U/L. Endoscopy revealed a gastric ulcer, which was positive for Helicobacter pylori and the patient was commenced on triple therapy. However, over the following 5 days, she developed features suggestive of a subacute cervical myelitis with weakness in the left upper limb and an emergent paraparesis. In addition, the patient reported paraesthesia and flexor spasms in the left hand and had impaired proprioception and vibration sense. She had no history of visual symptoms and examination revealed no eye or cranial nerves abnormalities. Magnetic resonance imaging (MRI) of the brain demonstrated hyperintensity at the floor of the fourth ventricle, in a region corresponding to the area postrema (Fig. 1b). Sagittal T2-weighted imaging of the spinal cord demonstrated a longitudinally extensive signal change: from the lower border of C3 to the upper border of C7 (Fig. 1c, d). Cerebrospinal fluid (CSF) analysis showed: an elevated protein level of 1.37 g/L, reduced glucose at 2.9 mmol/L (serum glucose was 6.8), raised white cell count at 8/lL (84 % mononuclear cells) and no oligoclonal bands. Polymerase chain reaction for a number of known demyelinating viruses including: herpes simplex, CMV and EBV were negative. No evidence of antibodies to aquaporin-4 were detected in the CSF, however, serology was positive: thereby fulfilling the diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD) [1]. Management consisted of intravenous methylprednisolone followed by a tapering dose of oral prednisolone. Although a radiological improvement was noted with steroids (Fig. 2), plasmapheresis was initiated due to an incomplete clinical recovery, however, treatment was stopped due to line sepsis. The patient subsequently received intravenous immunoglobulin and was noted to have an improvement in her weakness. Maintenance immunosuppression was commenced with azathioprine and no further relapses have occurred 9 months from the initial presentation. NMO is an inflammatory demyelinating disorder of the central nervous system characterised by acute episodes of optic neuritis and transverse myelitis [1, 2], this case provides a number of learning points. Firstly, the AQP4 antibodies in this case were positive in the serum, whilst no evidence of anti-AQP4 antibodies was found in the CSF. It has been shown that the concentration of the NMO antibodies in the plasma is 500 times more than in the CSF [2], which likely accounts for this finding. Furthermore, serum AQP4 antibodies have a specificity of over 95 % and sensitivity of 73 % for NMO [1, 3]. In addition, the hypoglycorrhachia and raised CSF protein seen in this case have previously been reported in NMO where CSF findings may mimic bacterial meningitis during a relapse [4]. Secondly, the elevated CK level was an unexpected finding in this case. While AQP4 water channels are strongly expressed in astrocyte foot processes, they are also found in the fast twitch fibres of skeletal muscles [5, 6], which likely explains the CK level in this case. In a case series of 733 patients with confirmed NMO, raised CK levels were found in 0.4 % [7]. To the best of our & Tarig Mohammed Abkur tarig1982@hotmail.com
تدمد: 1432-1459
0340-5354
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_________::c1cf4d85282054a8efa94dbc3ab99997
https://doi.org/10.1007/s00415-015-7980-4
حقوق: CLOSED
رقم الأكسشن: edsair.doi...........c1cf4d85282054a8efa94dbc3ab99997
قاعدة البيانات: OpenAIRE