Nedia Ben Achour

Anti-Ma2-encephalitis in a 2 year-old child: A newly diagnosed case and literature review

BACKGROUND Anti-Ma2-associated encephalitis is a rare paraneoplastic neurological syndrome characterized by isolated or combined limbic, diencephalic, or brainstem dysfunction. It is rarely reported in children. CASE STUDY We describe the clinical data of a 2-year-old girl referred to our department for refractory focal seizures associated with fever, followed by behavioural changes, speech disturbances and confusional episodes. Brain magnetic resonance imaging (MRI) showed left temporoparietal brain involvement. Haematological, biochemical and infectious evaluations were unremarkable. Autoimmune encephalitis was suspected. Paraneoplastic antibodies tests showed positive results for anti-Ma2 antibodies. Screening for underlying tumour was negative. Immunomodulatory treatment was administrated. The patient showed improvement of vigilance and behaviour. However, she kept refractory epilepsy. CONCLUSION Although poor response to immunotherapy, early diagnosis and appropriate treatment of this disorder may prevent irreversible sequelae.

Mixed movements disorders as an initial feature of pediatric lupus

UNLABELLED Systemic lupus erythematosus (SLE) is an immunologic disease of the early adulthood. In children, SLE is rare and neurological onset is uncommon. We report on an observation of pediatric lupus in heterozygous twins revealed by mixed movement disorders. CASE REPORT An 8-year-old boy, born to non consanguineous parents, with a family history of depression and a personal history of macular eruption, inflammatory polyarthralgias and a recurrent angina presented with acute movement and mood disorders. Neurological exam showed mild generalized choreic movements with motor and vocal tics. Antinuclear antibodies were positive. Brain MRI was normal. One year after, his twin brother presented with the same features. DISCUSSION Movement disorders are described in pediatric lupus but are unusual as inaugural features of the disease. In SLE, movement disorders such as chorea are usually reported. However, Tics seem to be exceptional. Pathophysiology remains unclear. Early onset and familial form support genetic implication in the pathogenesis of lupus. CONCLUSION Immune-mediated movement disorders such as in SLE are an established cause of acute movement disorders in child.

Letter to the editor: “A case of Guillain-Barré syndrome with meningeal irritation”

We have read with great interest the case report published by Ashikari Y et al. ûA case of Guillain-Barré syndrome with meningeal irritationý in Brain and Dev 2016 [1]. The authors report an atypical presentation of Guillain-Barré syndrome (GBS) with meningeal irritation in a 5-year-old girl. This interesting case proves that meningeal involvement in GBS is uncommon and may be misleading for clinicians. We would like to share our viewpoint based on our experience. A 2 year-old previously healthy girl was referred to our department for gait disorders after a benign fall with spinal trauma. Neurological examination showed a severe and painful stiff neck with retrocollis, tetraparesis and generalized areflexia. Spinal injury was suspected but surprisingly, no traumatic lesions were noticed on spinal MRI. Gadolinium images revealed enhancement of medullary cone and cauda equina. CSF analysis showed high proteins (77 mg/dl) and normal cell count (5/ml). Neurophysiological studies were compatible with severe acute motor axonal neuropathy (AMAN). The IgG and IgM anti gangliosides antibodies (GM1, GM2, GM3, GD1a, GD1b, GT1b, GA1b) were negative. The patient received intravenous immunoglobulines (0.4 g/kg/j for 5 days). Neck stiffness improved 2 weeks after treatment and the child had full recovery within 10 months (Hughes score:1 and MRC sum: 58/ 60). Therefore, the onset circumstances and the atypical clinical presentation may be confusing as in our patient [2]. Atypical signs of GBS including meningeal irritation are uncommon and more frequent in the pediatric population [2,3]. Underlying mechanism of meningeal irritation remains unclear and seems related to radiculitis. Such a case supports findings depicted by Ashikari Y et al., but singularity in our patient corresponds to the severity of neck stiffness. Therefore, we believe that these case reports by Ashikari Y et al., us and others incite to broaden the etiologies of meningeal irritation and to consider the spinal MRI as a useful tool in the atypical pediatric GBS [2–5]. Once again, we would like to thank our colleagues for sharing their interesting clinical experience.

Pediatric Multiple Sclerosis in Tunisia: A Retrospective Study over 11 Years

Introduction Pediatric multiple sclerosis (pMS) is a rare demyelinating disorder with an onset before the age of 18 years. In this study, we aimed to investigate the characteristics of pMS in Tunisian children. Patients and Methods We conducted a retrospective study over 11 years (2005–2016) including all patients diagnosed with pMS according to the International Pediatric Multiple Sclerosis Study Group (IPMSSG) criteria of 2012 and followed up in a tertiary care research center. Epidemiological, clinical, neuroimaging, laboratory, and therapeutic data were collected and analyzed. Results There were 21 patients. The male-female ratio was 1 : 3. Mean age at onset was 11 years (range: 3–17 years). Three patients had type 1 diabetes. Polyfocal presentation was preponderant (81%) with motor dysfunction in 57% of patients. Paroxysmal dystonia was noticed in 24%. All patients were diagnosed with relapsing-remitting form. Interferon beta was prescribed in 80% with a reduction of annual relapse rate. Conclusion The annual incidence of pMS in Tunisian children aged below 18 years could be estimated as 0.05 per 100,000. Singular features in our cohort were the frequent association with type 1 diabetes and the increased occurrence of dystonia. Greater awareness of pMS may be helpful to improve management strategies of children and their families.