Magnetic resonance imaging findings in children with spasmus nutans

Background Spasmus nutans (SN) is a rare pediatric ophthalmologic syndrome characterized by nystagmus, head bobbing, and abnormal head positioning. Historically, SN has been associated with underlying optic pathway gliomas (OPG); however, evidence of this association is based primarily on a small number of isolated case reports. Prior retrospective analyses have found the rate of OPG to be <2%, but these studies only intermittently used neuroimaging with computed tomography, which has limited sensitivity for detection of small lesions in the optic pathway. The purpose of this study was to investigate the association of SN with intracranial abnormalities, particularly OPG, using magnetic resonance imaging of the brain and orbits. Methods Neuroradiology databases at three institutions spanning January 2010 to May 2016 were queried for examinations ordered for evaluation of SN; MRI examinations of the brain and/or orbits were included and evaluated for OPG and other structural abnormalities. Medical records were reviewed to confirm a diagnosis of SN, presence of other underlying neurological disease, or preexisting diagnoses. Results A total of 40 patients with eligible MRI examinations were identified. None had optic nerve pathway gliomas. Two children had optic nerve hypoplasia; no other patients had optic pathway abnormalities. None had intracranial or orbital masses. MRI examinations were normal in 25 patients. Conclusions This series represents the largest collection of MRI examinations for SN in the literature to date and shows no association between OPG and SN. In children presenting with SN but no other findings suggesting OPG or neurological abnormalities, neuroimaging may not be required

A prospective study of treatments for adult-onset divergence insufficiency–type esotropia

Purpose To describe 10-week and 12-month outcomes following treatment for divergence insufficiency–type esotropia in adults. Methods In this prospective observational study, 110 adults with divergence insufficiency–type esotropia, with a distance esodeviation measuring 2Δ to 30Δ and at least 25% larger at distance than near, and binocular diplopia present at least “sometimes” at distance, were enrolled at 28 sites when initiating new treatment. Surgery, prism, or divergence exercises/therapy were chosen at the investigator’s discretion. Diplopia was assessed at enrollment and at 10-week and 12-month outcome examinations using a standardized diplopia questionnaire (DQ). Success was defined as DQ responses of “rarely” or “never” when looking straight ahead in the distance, with no alternative treatment initiated. Results Of the 110 participants, 32 (29%) were prescribed base-out prism; none had received prior treatment for esotropia. Success criteria were met by 22 of 30 at 10 weeks (73%; 95% CI, 54%-88%) and by 16 of 26 at 12 months (62%; 95% CI, 41%-80%). For the 76 (68%) who underwent strabismus surgery (82% of whom had been previously treated with prism), success criteria were met by 69 of 74 at 10 weeks (93%; 95% CI, 85%-98%) and by 57 of 72 at 12 months (79%; 95% CI, 68%-88%). Conclusions In this study cohort, both base-out prism as initial therapy and strabismus surgery (usually following prism) were successful in treating diplopia for most adults with divergence insufficiency–type esotropia when assessed during the first year of follow-up

Results Of Performing Extraocular Muscle Surgery On Patients Diagnosed With Myasthenia Gravis

The purpose of this study was to report the postoperative results of strabismus surgery on patients with stable myasthenia gravis (MG)

Pediatric Optic Neuritis - MOG/NMO/LP On All, or Some (Extensive Work-Up)

There is a wide differential for pediatric optic neuritis (ON) and the diagnostic workup for underlying etiologies needs to be carefully considered. Furthermore, bedside clinical pearls used to narrow the differential in adult ON don't always apply in pediatric ON. Children with ON are more likely to present with features such as optic nerve head edema, absence of pain, severe initial vision loss, and bilateral involvement, which would all be considered atypical for adult patients. Obtaining multiple imaging studies and lumbar puncture in adults with ON is routine, but in children these tests may be more controversial as sedation is required for the youngest patients. With the now wide availability of NMO and MOG antibody serum testing, many cases of pediatric ON associated with these disorders can be diagnosed with routine bloodwork, but these tests can take 10-14 days to return

Pediatric Optic Neuritis (Abstract)

LEARNING OBJECTIVES: 1. Describe the clinical characteristics and outcomes of optic neuritis in children. 2. Formulate a diagnostic workup for a pediatric patient with optic neuritis. 3. Describe the outcomes associated with anti-aquaporin-4 and anti-myelin oligodendrocyte glycoprotein antibody positivity in pediatric optic neuritis.KBDidiopathicopticneuriti

A Sad Story - Slides

A healthy 6-year-old boy had a two-week history of progressive headaches associated with nausea and vomiting. An ER diagnosed allergic rhinitis, treated with augmentin and steroids, however his symptoms worsened. At a second ER he was febrile to 103F. A classmate was recently diagnosed with Erlichiosis, and our patient was given; doxycycline. Brain MRI with and without contrast was normal. CBC was normal. A lumbar puncture revealed 286; WBC (75%lymphs) , 1 RBC, protein 65, glucose 24, negative gram stain. He was diagnosed with aseptic meningitis. Blood culture, CSF culture, CMV, EBV, Arbovirus, RMSF, Erlichia chaffeensis, Bartonella, and West Nile virus were; negative. He developed binocular horizontal diplopia. Neuro-ophthalmology was consulted. Afferent visual function was normal, extraocular motility was full, he had horizontal gaze-evoked nystagmus, an alternating; esotropia, and mild bilateral disc edema. Diagnosis was mild sixth nerve palsy from elevated ICP in the setting of; meningitis. Neurologic examination was otherwise unremarkable. Head CT showed mild communicating; hydrocephalus. Repeat LP showed opening pressure 33cmH2O, WBC 366 (60% lymphs), 4 RBC, protein 180, glucose 4, negative Cryptococcus, VDRL, HIV, HSV, TB by PCR. IV ceftriaxone and acyclovir were initiated. MRI; brain and spine revealed leptomeningeal enhancement and hydrocephalus. His sixth nerve palsies worsened. He developed bowel and bladder incontinence, and clincally deteriorated. Rifampin, isoniazide, pyrazinamide, and; ethambutol were initiated, with vancomycin and steroids. A right EVD was placed for hydrocephalus. Quantiferon Gold, urine and blood histoplasma antigen were negative. He began complaining of vision loss. Repeat head CT revealed dilation of the left lateral ventricle, and a left EVD was placed. He was intubated and sedated. Amphotericin was added. At this point the patient was covered for bacterial, viral, TB, and fungal meningitis. His mother noted he was exposed to well water and frequently played outside in the dirt.SMcomplicationsofinfections; VBintracranialinfection

A Sad Story - Video

A healthy 6-year-old boy had a two-week history of progressive headaches associated with nausea and vomiting. An ER diagnosed allergic rhinitis, treated with augmentin and steroids, however his symptoms worsened. At a second ER he was febrile to 103F. A classmate was recently diagnosed with Erlichiosis, and our patient was given; doxycycline. Brain MRI with and without contrast was normal. CBC was normal. A lumbar puncture revealed 286; WBC (75%lymphs) , 1 RBC, protein 65, glucose 24, negative gram stain. He was diagnosed with aseptic meningitis. Blood culture, CSF culture, CMV, EBV, Arbovirus, RMSF, Erlichia chaffeensis, Bartonella, and West Nile virus were; negative. He developed binocular horizontal diplopia. Neuro-ophthalmology was consulted. Afferent visual function was normal, extraocular motility was full, he had horizontal gaze-evoked nystagmus, an alternating; esotropia, and mild bilateral disc edema. Diagnosis was mild sixth nerve palsy from elevated ICP in the setting of; meningitis. Neurologic examination was otherwise unremarkable. Head CT showed mild communicating; hydrocephalus. Repeat LP showed opening pressure 33cmH2O, WBC 366 (60% lymphs), 4 RBC, protein 180, glucose 4, negative Cryptococcus, VDRL, HIV, HSV, TB by PCR. IV ceftriaxone and acyclovir were initiated. MRI; brain and spine revealed leptomeningeal enhancement and hydrocephalus. His sixth nerve palsies worsened. He developed bowel and bladder incontinence, and clincally deteriorated. Rifampin, isoniazide, pyrazinamide, and; ethambutol were initiated, with vancomycin and steroids. A right EVD was placed for hydrocephalus. Quantiferon Gold, urine and blood histoplasma antigen were negative. He began complaining of vision loss. Repeat head CT revealed dilation of the left lateral ventricle, and a left EVD was placed. He was intubated and sedated. Amphotericin was added. At this point the patient was covered for bacterial, viral, TB, and fungal meningitis. His mother noted he was exposed to well water and frequently played outside in the dirt.SMcomplicationsofinfections; VBintracranialinfection

Pediatric Optic Neuritis (Slides)

LEARNING OBJECTIVES: 1. Describe the clinical characteristics and outcomes of optic neuritis in children. 2. Formulate a diagnostic workup for a pediatric patient with optic neuritis. 3. Describe the outcomes associated with anti-aquaporin-4 and anti-myelin oligodendrocyte glycoprotein antibody positivity in pediatric optic neuritis

Pediatric Optic Neuritis (Video)

LEARNING OBJECTIVES: 1. Describe the clinical characteristics and outcomes of optic neuritis in children. 2. Formulate a diagnostic workup for a pediatric patient with optic neuritis. 3. Describe the outcomes associated with anti-aquaporin-4 and anti-myelin oligodendrocyte glycoprotein antibody positivity in pediatric optic neuritis.KBDidiopathicopticneuriti