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Purpose To assess the current burden of ocular toxocariasis (OT) and to gain knowledge regarding the diagnostic and treatment practices used in the ophthalmologic community in the United States. Design Web-based, cross-sectional survey. Participants Subspecialty ophthalmologists who are currently practicing in the United States. Methods An electronic survey was sent to 3020 ophthalmologic subspecialists belonging to the American Uveitis Society (AUS), the American Society of Retina Specialists (ASRS), or the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) to capture demographic, clinical, diagnostic, and treatment data on patients with OT seen in their practices between September 2009 and September 2010. Main Outcome Measures The demographic, epidemiologic, and clinical characteristics of each reported patient with OT. Results A total of 159 patients with OT were reported by 559 respondents (19%). The median patient age was 11.5 years (range, 1–66 years). Seventy-two patients (45%) with OT lived in the Southern region of the United States. Thirty-one (69%) of 45 patients with OT owned a dog or cat. Vision loss was reported in 46 (85%) of 54 patients with OT; 32 (71%) of 45 patients had permanent vision loss, 13 patients (29%) had temporary vision loss, and duration of vision loss was unreported for 1 patient. Of the 32 patients with OT with permanent vision loss, 30 (94%) had a subretinal granulomatous mass/scar, peripheral granuloma with traction bands, or posterior pole granuloma noted on ophthalmologic examination. Subretinal granulomatous mass/scar, vitritis, and scotoma were the most common ophthalmologic signs found on examination of patients with OT. Conclusions Ocular toxocariasis continues to occur in the United States, where it affects mainly children and causes permanent vision loss in many patients. Healthcare professionals should counsel patients and their family members about prevention strategies in an effort to decrease infection rates and morbidity due to Toxocara . Further improvement of diagnostic and treatment tools is needed to assist ophthalmologists in treating patients with OT. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Objective To describe a young girl in whom stimulus sensitive spasms were a diagnostic clue to the underlying glycine receptor antibody mediated progressive encephalomyelitis with rigidity and myoclonus (PERM) which has been described mainly in adults. Methods We illustrate the clinical data from the case-notes with a video recording of stimulus-sensitive spasms. Results A previously well 15-year-old girl presented with a traumatic head injury after falling down the stairs. The noise from a doorbell had triggered a severe muscle spasm causing the fall. She sustained a complex skull fracture with coup-contrecoup brain injury. She had previously been evaluated over five months for stimulus-sensitive whole body spasms with axial hyperextension without impairment of consciousness. She also had daily stimulus-sensitive myoclonic jerks, headaches and subtle cognitive impairment over the preceding year. Whilst on the paediatric intensive care unit, she was noted to have spontaneous and stimulus-sensitive muscle spasms (EMG duration 360–680 ms with no EEG correlate). She also developed generalised tonic clonic seizures, which were treated with Levetiracetam. A diagnosis of PERM was considered and glycine receptor antibodies were found to be strongly positive in serum and CSF. VGKC-complex antibodies were also weakly positive but GAD and NMDAR antibodies were negative. Oligoclonal bands were positive in CSF but negative in serum. Paraneoplastic antibodies were negative. Brain MRI showed contusions in the right hemisphere. Body imaging did not identify any tumours. Glycine receptor gene analysis is awaited. Her spasms have improved with a residual mild startle response to touch or loud auditory stimuli. She has a residual left hemiparesis. She was not treated with immunomodulation but will be followed up, and immunotherapy considered if she does not continue to improve and antibodies persist. Conclusion Glycine receptor antibody-mediated progressive encephalomyelitis with rigidity and myoclonus can present in children as stimulus-sensitive muscle spasms.

Background In patients with glycine receptor antibodies (GlyRAb), a range of neurological syndromes that includes Stiff Person Syndrome (SPS) and its more severe variant Progressive Encephalitis with Rigidity and Myoclonus (PERM) have recently been reported in a predominantly adult cohort (Carvajal-Gonzalez et al., Brain 2014;137(Pt 8):2178–92). Objectives To report the clinical features and outcome of children with GlyR-Ab. Methods Cases were identified from samples sent to the Nuffield Department of Clinical Neurology, Oxford for a range of neuronal surface antibody testing. Results Six girls with a mean age of 8 years (median 7.5; range 1–15) were identified (2 London, 1 Oxford, and 3 Australia). The children presented with refractory focal seizures (n=2), or cognitive/behavioural changes (n=4) of whom two had tremor and gait abnormalities and one features of PERM. During the course of the illness, seizures featured in 5 patients and cognitive/behavioural changes in all. Electroencephalogram (EEG) during the illness revealed features of encephalopathy (n=3) and focal/multifocal epileptiform discharges (n=2). Two children had early imaging features of brain inflammation, and in another 2, global atrophy were identified on serial imaging despite normal acute scans. GlyR-Abs were detected in the serum (n=6) and/or CSF (n=2). Co-occurrence of low levels ( Conclusions The paediatric phenotype is predominated by female sex, seizures, and, where PERM occurs, it is associated with additional features. The clinical relevance of GlyR antibodies needs to be determined, to help guide therapies.