Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord that can cause loss of sensation and muscle spasticity. Guillain-Barre syndrome (GBS) is a postinfection autoimmune disease, classified as an acute polyneuropathy. This report describes the emergency admission of a 6-year-old girl presenting with sudden pallor and pain in both lower limbs. The patient’s reflexes were normal, as were the results of her sonography, radiography and biochemical tests; however, spinal MRI revealed extensive compartmentalised syringomyelia extending from C2 to T3. A sensory and motor nerve conduction study revealed a demyelinating type motor polyneuropathy which, along with positiveMycoplasma pneumoniaetest, was suggestive of GBS. Intravenous immunoglobulin infusion showed excellent results. In conclusion, we report a rare paediatric case of syringomyelia coexisting with GBS. It is important to bear in mind the possibility of other coexisting diseases even if MRI reveals definitive characteristics of another condition.
Idiopathic intracranial hypertension (IIH) is a rare neurological syndrome characterized by increased intracranial pressure (>28 cm H₂O in children), in the absence of intracranial space-occupying lesions, and with normal cerebrospinal fluid (CSF) composition. Optic perineuritis (OPN) is an orbital inflammatory disease confined to the optic nerve sheath. We report a case of concurrent IIH and bilateral OPN in a previously healthy child. A 13-year-old male, with normal body mass index, presented with diplopia, visual blurriness without ocular pain, and occipital headache. Neurological examination was notable only for right sixth nerve palsy. Ophthalmological assessment revealed bilateral papilledema. Brain and spinal cord magnetic resonance imaging (MRI) and computed tomography (CT) scans demonstrated bilateral optic nerve perineuritis. Lumbar puncture showed an opening pressure of 98 cm H₂O, with normal CSF composition. The patient was treated with acetazolamide and corticosteroids, resulting in marked clinical improvement and complete restoration of vision at two-month follow-up. The concurrent occurrence of IIH and bilateral OPN is extremely rare, particularly in pediatric patients. To our knowledge, this is the first reported case in a child. Recognition of this dual pathology is essential for timely diagnosis and effective treatment.
27 June 2026 in Multiple Sclerosis and Related Disorders
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a distinct immune-mediated demyelinating central nervous system disorder, frequently presenting as optic neuritis in children and adolescents. Conversely, aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) is an astrocytopathy characterized by more severe relapses and greater residual visual impairment. This scoping review, conducted according to PRISMA-ScR and JBI methodology, mapped evidence from 23 studies identified across PubMed and Scopus over the last decade. The objective was to examine the epidemiology, clinical presentation, imaging, serological findings, therapeutic strategies, and visual outcomes of these conditions in pediatric populations. Results indicate that pediatric MOGAD-associated optic neuritis often presents with optic disc swelling and bilateral anterior optic nerve involvement with perineural enhancement on MRI, typically resulting in reversible visual loss. The 2023 MOGAD diagnostic criteria show high performance in children. In contrast, AQP4-associated optic neuromyelitis more commonly involves the posterior optic nerve and chiasm, leading to poorer visual outcomes. Regarding treatment, maintenance intravenous immunoglobulin (IVIG) reduces relapse risk in selected MOGAD cases, while B-cell-depleting regimens are primarily used for relapse prevention in AQP4-positive NMOSD. In conclusion, pediatric optic neuritis in MOGAD and AQP4-associated optic neuromyelitis are distinct phenotypes with differing pathobiology and therapeutic requirements. Early serological confirmation and individualized immunotherapy are crucial for preventing relapses and preserving functional vision in pediatric patients.