Ibtihel Rebai

Postencephalitic parkinsonism and selective involvement of substantia nigra in childhood

Parkinsonism is a rare complication of encephalitis in childhood. Association to an isolated involvement of substantia nigra is exceptional. Mechanisms of nigral cells neurotropism remain hypothetic. We report on three children presenting with postencephalitic parkinsonism and selective involvement of substantia nigra, with literature review and we discuss pathogenic mechanisms.

Severe dysautonomia as a main feature of anti-GAD encephalitis: Report of a paediatric case and literature review

INTRODUCTION Anti-glutamic acid decarboxylase (anti-GAD65) antibodies are a rare cause of autoimmune encephalitis. This entity is mainly recognized in adults and very few cases were reported in children. We report on a paediatric case of anti-GAD encephalitis with severe presentation and uncontrollable dysautonomia. CASE STUDY A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal. Repeat screening for antineuronal antibodies showed negative results for anti-NMDA receptor antibodies but positive results for anti-GAD65 with a low positivity of anti-Ma2 antibodies. Although a transient improvement was noticed after immunomodulatory treatment, the patient developed severe intractable autonomic imbalance including dysrythmia, alternating bradycardia/tachycardia, hypotension/hypertension, hypothermia/hyperthermia and hyperhidrosis. She deceased six months after onset. CONCLUSION Our report intends to raise awareness of autoimmune encephalitis with anti-GAD65 antibodies which may involve extralimbic brain regions and manifest with fatal dysautonomia. We highlight the need for prompt diagnosis and aggressive management for this underdiagnosed entity in children.

Comment on ‘Acute flaccid myelitis in childhood: a retrospective cohort study’

Dear Editor, We have read with great interest the paper by Andersen et al., ‘Acute flaccid myelitis in childhood: a retrospective cohort study’, in the European Journal of Neurology (June 2017) [1]. This retrospective cohort study reports eight children with acute flaccid paralysis with extensive longitudinal myelitis and preferential involvement of grey matter. The newly recognized entity termed ‘acute flaccid myelitis’ (AFM) is uncommon and has been related mainly to nonpolio enteroviruses. We would like to share our viewpoint based on our experience. In January 2016, a 4-year-old girl with no family or medical history was admitted for progressive weakness and pain of the lower limbs over 2 days. She complained of gastric pain 1 week earlier. Neurological examination showed a flaccid paraplegia with absent deep tendon reflexes and plantor flexor. No sensitive or cranial nerve involvement was noticed. Guillain Barr e syndrome (GBS) was initially suspected. Electroneuromyographic study showed prolonged F-wave latencies. Five days after onset, the child had brisk tendon reflexes, Babinski sign and urinary retention. Brain and spinal magnetic resonance imaging (MRI) was performed revealing pontine increased signal and spinal abnormalities confined to the central grey matter on T2-weighted images extending from C5 to the terminal cone without gadolinium enhancement (Fig. 1). Routine biological tests were normal. Cerebrospinal fluid (CSF) analysis showed a pleocytosis (25 cells/ll) with normal protein level (0.18 g/l). Viral and bacterial serological analysis of herpes simplex virus, Epstein–Barr virus, varicella zoster virus, cytomegalovirus, rubella virus, hepatitis B and C, mycoplasma, West Nile virus, human immunodeficiency virus, lyme disease and syphilis and CSF polymerase chain reaction for enterovirus were negative. Immunological tests (anti nuclear, anti neuromyelitis optica, anti rheumatoid factor and anti Sm antibodies) were negative. AFM was diagnosed and the patient received intravenous immunoglobulins (IVIG) (0.4 g/kg/day for 5 days) with a full recovery. Two months later, cerebral and spinal MRI showed persistent pontine and spinal abnormalities. This observation highlights the rarity of AFM as a differential diagnosis of GBS and transverse myelitis with a unique feature involving electively the spinal grey matter and residual neurological deficits in 90% of patients [1–3]. Awareness of this disorder especially in countries where polio has been eradicated may lead to more rapid diagnosis and avoid delay of treatment, mainly with IVIG, which

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.