L E T T E R T O T H E E D I T O R
Intractable hiccup and vomiting, neuropathic pruritus and tonic spasms in a case of neuromyelitis optica spectrum disorder
Hicham El Otmani
•Fatima Dany
•Bouchra El Moutawakil
•Mohammed Abdoh-Rafai
•Ilham Slassi
Received: 22 September 2014 / Accepted: 22 December 2014 ÓBelgian Neurological Society 2015
Keywords Neuromyelitis optica Neuropathic pruritus Hiccups vomiting Tonic spasms
Introduction
Neuromyelitis optica (NMO) has been traditionally descri- bed as the association of optic neuritis and longitudinally extensive transverse myelitis (LETM) [1]. Identification of aquaporin-4 antibody (AQP-4) has deeply changed the concept of this disease and allowed identification of cases beyond this classical phenotype [1]. Indeed, clinical and MRI analysis of larger cohorts in the last few years, allowed the description of various aˆ%
aatypical aˆ%« clinical symptoms, as a syndrome of intractable hiccups nausea and vomiting (IHNV) [2] or more recently, painful tonic spasms (PTS) [3] and neuropathic pruritus (NP) [4].
We report a case of LETM associated with AQP-4 anti- bodies, preceded by IHNV and NP and followed by PTS. To our knowledge, the association of these three unusual symp- toms in the same patient has never previously been reported.
Case report
A 45-year-old man was admitted in September 2010 after 9 weeks of severe vomiting and hiccups (resulting in a
weight loss of 11 kg) and 5 weeks of acute onset intense and continuous pruritus that affected the right suprascap- ular and subclavicular area without skin lesion or trigger factors. Paraclinical assessment including gastric endos- copy was normal and symptomatic treatment had no effect.
Two days before hospitalization in Neurology department, he developed a rapid onset of right hand and legs paralysis and sphincter disturbances. MRI showed myelitis between C2–C5 (Fig.
1a) with peculiar involvement of C3–C4posterior horns (Fig.
1b) and lesion in the area postremawith contrast enhancement (Fig.
1a, c). The CSF testingrevealed 20 lymphocytes, normal protein content and oli- goclonal bands were absent. Serum and CSF AQP-4 anti- bodies returned as positive. Ophthalmological assessment including visual evoked potentials was normal. Six days after starting methylprednisolone infusion, hiccups and vomiting disappeared. Pruritus improved significantly, leaving a cutaneous hyperesthesia in the same area.
Treatment with oral corticosteroids and monthly cyclo- phosphamide pulse was started.
Four weeks later, when the patient began a marked improvement in motor deficit, he presented several daily episodes of paroxysmal painful tonic muscle contraction, affecting right upper limb, lasting for 30 s (video 1). These symptoms disappeared a few days after treatment with carbamazepine at a dose of 500 mg per day. At last review in October 2014, he remained relapse free; including hic- cups, itching and spasms.
Discussion
In patients with definite NMO, optic neuritis and acute transverse myelitis usually have well-defined clinical characteristics [1]. The case described here, illustrates three
Electronic supplementary material The online version of thisarticle (doi:10.1007/s13760-014-0418-4) contains supplementary material, which is available to authorized users.
H. El Otmani (&)F. DanyB. El Moutawakil M. Abdoh-RafaiI. Slassi
Department of Neurology, Ibn Rochd Universitary Hospital, Casablanca, Morocco
e-mail: hichamotmani@hotmail.com
123
Acta Neurol Belg
DOI 10.1007/s13760-014-0418-4
less common features occurring in the same patient with NMO spectrum disorders.
IHN seem to be a common feature in NMO [2]. Misu and al. [2], reported that 20 % of NMO patients have these symptoms, whereas none of the patients with mul- tiple sclerosis had similar complaints. As the case described here, the episodes often precede myelitis attack, resolved with methylprednisolone treatment and result from involvement of the area postrema, a vomiting reflex center at the floor of the fourth ventricle. In some cases, IHN and other symptoms suggesting brainstem lesions (vertigo, ataxia, dysarthria, ocular movement disorders and other cranial nerve involvement) can represent a relapse with no subsequent development of optic neuritis or myelitis [5].
PTS (or paroxysmal dystonia) is a paroxysmal tonic muscle contraction affecting one or more limbs that usually lasts for a few seconds. In Kim and al. study [3], the incidence of PTS was significantly higher in the patients with NMO (25 %) than in those with multiple sclerosis (3 %) or idiopathic acute transverse myelitis without AQP- 4 antibody (2.4 %). In our case, PTS occurred during recovery phase of myelitis and was very responsive to carbamazepine. These characteristics are similar to those reported in others case series [3].
Recently, Elsone et al. [4] reported that 12 out of 44 (27 %) patients who had myelitis as part of AQP4-A positive NMO or NMOSD experienced pruritus (or itch)
without a rash. This neuropathic pruritus was the initial symptom of myelitis in three patients, occurred a median of five days before the emergence of other sensory-motor symptoms. In our patient, pruritus was present 5 weeks before the onset of motor deficit and good correlate can be established between affected dermatomes (right C3, C4 and C5) and the posterior horns MRI lesions. The dorsal horn is an area rich in the gastrin-releasing protein receptor bear- ing neurons that mediate pruritus and may also be more AQP4 rich, so are therefore preferentially involved in NMO [4].
In summary, our case illustrates the possible occurrence of several unusual symptoms in the same NMOSD patient.
It also demonstrates that the IHNV and NP are correlated, respectively, with lesion of area posttrema and posterior horn, and may be warning signs indicating forthcoming myelitis attack.
Conflict of interest None.
References
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Fig. 1 aSagittal T2-weighted MRI of the cervical cord showing longitudinally extensive myelitis between C2 and C5baxial flair slice showing predominantly posterior horns lesions (arrow)cMRI showing an hyperintense lesion of the area postrema with contrast enhancement (arrow) Acta Neurol Belg
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