Rituximab, IVIg, and Tetracosactide (ACTH1-24) Combination Immunotherapy ("RITE-CI") for Pediatric Opsoclonus-Myoclonus Syndrome: Immunomarkers and Clinical Observations.

Opsoclonus-myoclonus syndrome (OMS) is a neuroinflammatory disorder with pervasive morbidity that warrants better treatments. Twelve children with moderate/severe OMS (total score 23 ± 6) who did not remit to multiple immunotherapies were evaluated for neuroinflammation in a case-control study using cerebrospinal fluid (CSF) lymphocyte subset analysis by flow cytometry, chemokine/cytokine analysis by enzyme-linked immunoadsorption assay (ELISA), and oligoclonal bands by immunofixation with isoelectric focusing. Observations made on empirical treatment with rituximab, IVIg, and tetracosactide combination immunotherapy (coined "RITE-CI") were analyzed. All of the patients tested for multiple inflammatory markers were positive; 75% had ≥3 CSF markers. Fifty percent had CSF oligoclonal bands; 58%, B cell expansion; and 50 to 100%, elevated concentrations of multiple chemokines and neuronal/axonal marker neurofilament light chain. After RITE-CI, total score dropped significantly in the group (-85%, p < 0.0001) from moderate to trace, and by 2 to 4 severity categories in each patient. The 24-week schedule was well tolerated and clinically effective for moderate or severe OMS, as were other schedules. RITE-CI is feasible and effective as rescue therapy and presents an initial option for children with moderate/severe OMS. Though preliminary, the schedule can be adjusted to patient severity, propensity for relapse, and other factors.

Visual and neurologic deterioration in otogenic lateral sinus thrombosis: 15 year experience.

OBJECTIVES: Otogenic lateral sinus thrombosis is a rare complication of acute otitis media whose clinical presentation has changed with the early use of antibiotics. The aim of this study was to analyze the changing clinical signs, vaccination status, therapeutic management and outcome of these patients. METHODS: Retrospective chart review of 10 children treated with otogenic lateral sinus thrombosis in a tertiary level teaching hospital in Budapest, Hungary, from January 1998 till August 2013. RESULTS: Patients were divided into Early and Late presenting groups. In the Early presenting group, sepsis developed within one week after the onset of acute otitis media. At admission otological symptoms were predominant. The Late presenting group experienced acute otitis media several weeks prior to presentation and in this group neurologic symptoms dominated the clinical picture at admission. All patients received antibiotics. Eight of them were also treated with low molecular weight heparin. All children underwent cortical mastoidectomy. After surgery, the clinical signs of elevated intracranial pressure transiently worsened. This manifested as progression of papilledema in seven children, causing severe visual disturbance in two cases. After medical treatment and serial lumbar punctures all patients except one recovered. This child has permanent visual acuity failure of 0.5D unilaterally. At one year follow up complete and partial recanalization were noted in five and two patients, respectively. CONCLUSIONS: After mastoidectomy the signs of elevated intracranial pressure can transiently worsen, papilledema can progress. Daily bedside monitoring of visual acuity and regular ophthalmoscopy with neurologic examination is recommended during hospitalization. Close follow up is advised up to one year. When the dominant sinus is occluded, the clinical scenario is more protracted and severe.

Timely recanalization of lateral sinus thrombosis in children: should we consider hypoplasia of contralateral sinuses in treatment planning?

The objective of this study was to evaluate long-term clinical and radiological outcomes in children treated with lateral sinus thrombosis secondary to acute mastoiditis considering also contralateral sinus hypoplasia. This study was a retrospective chart review, conducted in tertiary pediatric hospital. Medical reports of eight children with acute mastoiditis and lateral sinus thrombosis from 1998 to 2011 were examined in terms of therapy, clinical recovery and radiological proof of lateral sinus recanalization. Three children presented hypoplasia of contralateral venous drainage system. Condition of sinuses was regularly monitored with MRI. Otologically and neurologically, all children recovered fully. All received antibiotics; six received additional low molecular weight heparin therapy. Mastoidectomy was performed on six cases. Incision and thrombectomy were applied in the other two, one involving internal jugular vein ligation. This latter case presented also contralateral venous hypoplasia; visual impairment proved permanent. The other two children with contralateral sinus hypoplasia recovered fully after steroid, dehydration and low molecular weight heparin therapy. Recanalization occurred in all children except the one with internal jugular vein ligation, in whom good collateral circulation was observed. There were no bleeding complications. Anatomical variations of cerebral venous drainage system can frequently be observed and should be considered in treatment planning. Mastoidectomy with antibiotics and additional low molecular weight heparin treatment is a safe, promising alternative to thrombectomy and internal jugular vein ligation in children with lateral sinus thrombosis following acute mastoiditis, also having contralateral sinus hypoplasia. Recanalization can be expected within two to five months.

[Acute transverse myelitis in childhood]. / Akut myelitis transversa gyermekkorban.

We reviewed the medical history, clinical signs, imaging studies, laboratory data and treatment effectiveness of our 10 patients presented with acute idiopathic transverse myelitis. We used the criteria of the Transverse Myelitis Consortium Working Group (2002). So we excluded all those cases by whom the cause of the inflammation could be detected (e. g. direct viral inflammatory disease, systemic autoimmune disease). Age of the patients at disease onset ranged from 3 to 15 years. The first clinical signs were pain in different locations, and urinary retention. Paraparesis or plegia reached its maximum within five days. By all patients spinal MRI and lumbar puncture were performed at admission. These results were interpreted together with the clinical signs, and therapy was started immediately. We used methylprednisolon pulse therapy. Within 10-30 days the patients started to walk. We have followed the children for 1.5-13 years. Few residual clinical signs were observed: by one child left sided spastic monoparesis persisted, by the other right sided latent monoparesis was stated, and by one partial urinary incontinence persisted. By the control spinal MRI persisting signal changes or atrophy were detected just by those two children who had residual clinical signs. In the follow-up period no clinical relapse occured. Neither did the brain or spinal MRI show new lesions. The quick diagnosis and the immediately started therapy determine mostly the clinical outcome of these children. We hope that our long follow-up period can help in better understanding the disease even in adult patients. In the future we try to join multicenter clinical studies.