Visual Evoked Potential Characteristics and Early Diagnosis of Pelizaeus-Merzbacher Disease

Pelizaeus-Merzbacher Disease with PLP1 Exon 1 Duplication, Previously Misdiagnosed as Cerebral Palsy: a Case Report

“…PMD is a dysmyelinating disease, but peripheral nervous system can be preserved since proteolipid protein constitutes a small proportion of the total myelin protein [ 13 ]. In our case, electrodiagnostic studies such as H-reflex and F-wave showed normal latencies representing preservation of peripheral nervous system, although previous study investigated that electrodiagnostic studies including visual evoked potentials were grossly abnormal in PMD patients [ 14 ].…”

Pelizaeus-Merzbacher Disease with PLP1 Exon 1 Duplication, Previously Misdiagnosed as Cerebral Palsy: a Case Report

“…PMD is a dysmyelinating disease, but peripheral nervous system can be preserved since proteolipid protein constitutes a small proportion of the total myelin protein [ 13 ]. In our case, electrodiagnostic studies such as H-reflex and F-wave showed normal latencies representing preservation of peripheral nervous system, although previous study investigated that electrodiagnostic studies including visual evoked potentials were grossly abnormal in PMD patients [ 14 ].…”

Modeling the Chronic Loss of Optic Nerve Axons and the Effects on the Retinal Nerve Fiber Layer Structure in Primary Disorder of Myelin

“…The aberrant fVEP response in shp dogs is consistent with abnormal VEPs reported in demyelinating diseases 12 , 52 and also in PMD. 53 The VEPs measured in this study were elicited by flash stimulation. Studies using multifocal pattern stimulation have shown a VEP amplitude reduction precedes axon loss in patients after ON.…”

Visual Evoked Potentials in Pediatrics—Abnormal

“…[22][23][24] The third form of developmental defect involves delays or alterations in the course of brain maturation. The migrational disorders of the cortex include such disorders as lissencephaly, pachygyria, and polymicrogyria.…”

Pelizaeus-Merzbacher Disease with PLP1 Exon 1 Duplication, Previously Misdiagnosed as Cerebral Palsy: a Case Report

“…PMD is a dysmyelinating disease, but peripheral nervous system can be preserved since proteolipid protein constitutes a small proportion of the total myelin protein [ 13 ]. In our case, electrodiagnostic studies such as H-reflex and F-wave showed normal latencies representing preservation of peripheral nervous system, although previous study investigated that electrodiagnostic studies including visual evoked potentials were grossly abnormal in PMD patients [ 14 ].…”

Modeling the Chronic Loss of Optic Nerve Axons and the Effects on the Retinal Nerve Fiber Layer Structure in Primary Disorder of Myelin

“…The aberrant fVEP response in shp dogs is consistent with abnormal VEPs reported in demyelinating diseases 12 , 52 and also in PMD. 53 The VEPs measured in this study were elicited by flash stimulation. Studies using multifocal pattern stimulation have shown a VEP amplitude reduction precedes axon loss in patients after ON.…”

Visual Evoked Potentials in Pediatrics—Abnormal

“…[22][23][24] The third form of developmental defect involves delays or alterations in the course of brain maturation. The migrational disorders of the cortex include such disorders as lissencephaly, pachygyria, and polymicrogyria.…”

Pelizaeus-Merzbacher Disease with PLP1 Exon 1 Duplication, Previously Misdiagnosed as Cerebral Palsy: a Case Report

“…PMD is a dysmyelinating disease, but peripheral nervous system can be preserved since proteolipid protein constitutes a small proportion of the total myelin protein [ 13 ]. In our case, electrodiagnostic studies such as H-reflex and F-wave showed normal latencies representing preservation of peripheral nervous system, although previous study investigated that electrodiagnostic studies including visual evoked potentials were grossly abnormal in PMD patients [ 14 ].…”

Modeling the Chronic Loss of Optic Nerve Axons and the Effects on the Retinal Nerve Fiber Layer Structure in Primary Disorder of Myelin

“…The aberrant fVEP response in shp dogs is consistent with abnormal VEPs reported in demyelinating diseases 12 , 52 and also in PMD. 53 The VEPs measured in this study were elicited by flash stimulation. Studies using multifocal pattern stimulation have shown a VEP amplitude reduction precedes axon loss in patients after ON.…”

Visual Evoked Potentials in Pediatrics—Abnormal

“…[22][23][24] The third form of developmental defect involves delays or alterations in the course of brain maturation. The migrational disorders of the cortex include such disorders as lissencephaly, pachygyria, and polymicrogyria.…”