A Case of An Unusual Bell ’s Palsy Mimic
We discuss the case of a 5-year-old boy who presented with an isolated left-sided cranial nerve 7 palsy that was initially magnetic resonance imaging negative. Owing to continued symptoms, repeat magnetic resonance imaging was performed and showed a temporal bone encephalocele. A review of the differential diagnosis of cranial nerve 7 palsy, warning signs signaling the need for additional workup, and a discussion of temporal lobe encephaloceles is provided in this case report. It is important to recognize that structural lesions can closely mimic idiopathic Bell ’s palsy, despite initial negative imaging. (Source: Semina...
Source: Seminars in Pediatric Neurology - April 14, 2017 Category: Neurology Authors: Alexandria L. Lutley, Shannon M. Standridge Source Type: research
A case of an unusual Bell ′s Palsy mimic
We discuss the case of five-year-old male who presented with an isolated left-sided cranial nerve seven palsy that was initially MRI negative. Due to continued symptoms, repeat MRI was performed and showed a temporal bone encephalocele. A review of the differential diagnosis of cranial nerve seven palsy, warning signs signaling the need for additional workup and a discussion of temporal lobe encephaloceles is provided in the case report. It is important to recognize that structural lesions can closely mimic idiopathic Bell ′s palsy, despite initial negative imaging. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 14, 2017 Category: Neurology Authors: Alexandria L. Lutley, Shannon M. Standridge Source Type: research
Diplopia with Dural Fibrotic Thickening
Idiopathic hypertrophic pachymeningitis is a rare disorder of diffuse thickening of the cranial or spinal dura matter without an identifiable cause. Most common in adult males, idiopathic hypertrophic pachymeningitis typically presents with headache with or without varied associated focal neurologic deficits and findings of dural enhancement on MRI in a linear, nodular or combined pattern. As it is felt to be an autoimmune disorder, treatment with high dose corticosteroids is typically recommended, and without intervention, the course is usually progressive. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 14, 2017 Category: Neurology Authors: Brittany Brand, Dave Somers, Blake Wittenberg, Jessica Gautreaux, Stephen Deputy Source Type: research
The Terrible Summer of ‘52…when Polio struck our family
Based on information gathered from the Knutson Siblings (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 14, 2017 Category: Neurology Authors: Linda Knutson Strand Tags: Case Studies issue Source Type: research
The Curse of Apneic Spells
A 6-year-old girl had reduced fetal movements, numerous apneic spells, muscle hypotonia, and developmental motor delay. Her muscle biopsy tissue showed variation in myofiber diameters, small minicores by electron microscopy, and near-uniformity of type I fibers. Although no mutations were detected in RYR1, SEPN1, and DMPK genes, the RAPSN gene revealed one known mutation, p.Asn88Lys, from the mother, and one novel mutation, p.Cys366Gly, from the father. Life-saving pyridostigmine treatment suppressed her apneic spells and improved her motor development. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 13, 2017 Category: Neurology Authors: Josefine Radke, Mona Dreesmann, Michael Radke, Arpad von Moers, Angela Abicht, Werner Stenzel, Hans H. Goebel Source Type: research
A 20-Month-Old Girl With Fever, Seizures, Hemiparesis, and Brain Lesions Requiring a Diagnostic Brain Biopsy
We report a case of a 20-month-old girl with atypical presentation of a fairly common condition. She presented with acute onset of fever, seizures, and hemiparesis. Her cerebrospinal fluid showed total nucleated cells 10/mm3 with lymphocyte dominance. Polymerase chain reaction was negative for herpes simplex virus. Computed tomography of head showed a cerebral hemorrhage of the left frontal lobe and small subarachnoid hemorrhage in the right frontal region. Brain magnetic resonance imaging revealed multiple foci of hyperintensity in bilateral parietal lobes and right basal ganglia. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 13, 2017 Category: Neurology Authors: Satsuki Matsumoto, Eiyu Matsumoto Source Type: research
The Curse of Apneic Spells
A 6-year-old girl had reduced fetal movements, numerous apneic spells, muscle hypotonia and developmental motor delay. Her muscle biopsy tissue showed variation in myofiber diameters, small minicores by electron microscopy, and near-uniformity of type I fibers. While no mutations were detected in RYR1, SMN, and SMARD1 genes, the RAPSN gene revealed one known mutation, p.Asn88Lys, from the mother, and one novel mutation, p.Cys366Gly, from the father. Life-saving pyridostigmine treatment suppressed her apneic spells and improved her motor development. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 13, 2017 Category: Neurology Authors: Josefine Radke, Mona Dreesmann, Michael Radke, Arpad von Moers, Angela Abicht, Werner Stenzel, Hans H. Goebel Source Type: research
A 20-Month-Old Girl with Fever, Seizures, Hemiparesis and Brain Lesions Requiring a Diagnostic Brain Biopsy
We report a case of a 20-month-old girl with atypical presentation of a fairy common condition. She presented with acute onset of fever, seizures and hemiparesis. Her cerebrospinal fluid (CSF) showed total nucleated cells 10/mm3 with lymphocyte dominance. Herpes simplex virus (HSV) polymerase chain reaction (PCR) result was negative. Computed tomography (CT) of head showed a cerebral hemorrhage of the left frontal lobe and small subarachnoid hemorrhage in right frontal region. Brain MRI revealed multiple foci of hyperintensity in bilateral parietal lobes and right basal ganglia. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 13, 2017 Category: Neurology Authors: Sat suki Matsumoto, Eiyu Matsumoto Source Type: research
Disentangling How the Brain is “Wired” in Cortical (Cerebral) Visual Impairment
Cortical (cerebral) visual impairment (CVI) results from perinatal injury to visual processing structures and pathways of the brain and is the most common cause of severe visual impairment or blindness in children in developed countries. Children with CVI display a wide range of visual deficits including decreased visual acuity, impaired visual field function, as well as impairments in higher-order visual processing and attention. Together, these visual impairments can dramatically influence a child ’s development and well-being. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 10, 2017 Category: Neurology Authors: Lotfi B. Merabet, D. Luisa Mayer, Corinna M. Bauer, Darick Wright, Barry S. Kran Source Type: research
A Review of Visual and Oculomotor Outcomes in Children With Posterior Fossa Tumors
This article outlines the relevant visual anatomy affected by these tumors and reviews the visual and oculomotor outcomes associated with the following 3 most common tumor types —medulloblastoma, juvenile pilocytic astrocytoma, and ependymoma. The available data suggest that the rate of permanent vision loss is low (5.9%-8.3%), with patients having juvenile pilocytic astrocytoma demonstrating the best outcomes. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 10, 2017 Category: Neurology Authors: Crandall E. Peeler Source Type: research
Pediatric Optic Neuritis
Optic neuritis is rare in children in comparison to adults, but accounts for approximately 25% of pediatric acute demyelinating syndromes. Features of pediatric optic neuritis that differ from adults include a higher rate of bilaterality, poor visual acuity on presentation, and papillitis. Diagnostic work-up includes brain magnetic resonance imaging (MRI), lumbar puncture, and blood tests to exclude infectious and inflammatory disorders. Pediatric optic neuritis may occur following infection or vaccination, or in association with a systemic demyelinating process such as acute disseminated encephalomyelitis (ADEM), neuromye...
Source: Seminars in Pediatric Neurology - April 10, 2017 Category: Neurology Authors: Melinda Y. Chang, Stacy L. Pineles Source Type: research
Disentangling How the Brain is “Wired” in Cortical/Cerebral Visual Impairment (CVI)
Cortical/cerebral visual impairment (CVI) results from perinatal injury to visual processing structures and pathways of the brain and is the most common cause of severe visual impairment/blindness in children in developed countries. Children with CVI display a wide range of visual deficits including decreased visual acuity, impaired visual field function, as well as impairments in higher order visual processing and attention. Together, these visual impairments can dramatically impact upon a child ′s development and well-being. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 10, 2017 Category: Neurology Authors: Lotfi B. Merabet, D. Luisa Mayer, Corinna M. Bauer, Darick Wright, Barry S. Kran Source Type: research
Optic Pathway Gliomas Secondary to Neurofibromatosis Type 1
Children with neurofibromatosis type 1 frequently manifest optic pathway gliomas —low-grade gliomas intrinsic to the visual pathway. This review describes the molecular and genetic mechanism driving optic pathway gliomas as well as the clinical symptoms of this relatively common genetic condition. Recommendations for clinical management and descriptions of the newest imaging t echniques will be discussed. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 10, 2017 Category: Neurology Authors: Shannon Jeanine Beres, Robert A. Avery Source Type: research
The Impact of Visual and Oculomotor Outcomes in Children with Posterior Fossa Tumors
This article outlines the relevant visual anatomy affected by these tumors and reviews the visual and oculomotor outcomes associated with the three most common tumor types – medulloblastoma, juvenile pilocytic astrocytoma, and ependymoma. The available data suggest that the rate of permanent vision loss is low (5.9–8.3%), with juvenile pilocytic astrocytoma patients having the best outcomes. (Source: Seminars in Pediatric Neurology)
Source: Seminars in Pediatric Neurology - April 10, 2017 Category: Neurology Authors: Crandall E. Peeler Source Type: research
