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61 Possible Causes for Electromyogram Abnormal, Hypomyelination

  • Peripheral Neuropathy

    Magnetic resonance imaging showed cortical disturbances in addition to profound hypomyelination and cerebellar atrophy.[ncbi.nlm.nih.gov] […] asymmetric demyelinating-type peripheral neuropathy, and sural nerve biopsy documented reduced myelinated nerve fiber density with uniformly thin myelin sheaths, suggesting hypomyelination[ncbi.nlm.nih.gov] We describe a consanguineous girl exhibiting severe developmental regression, intractable epilepsy, polyneuropathy, and profound hypomyelination with cortical involvement.[ncbi.nlm.nih.gov]

  • Secondary Myopathy

    Congenital hypomyelinating neuropathies. It may be difficult to distinguish myopathy from peripheral neuropathy.[patient.info] Although changes seen on electromyography are not pathognomonic for any specific disease process, an abnormal electromyogram can indicate if a neuropathy or neuromuscular[aafp.org] MRI shows diffuse cerebral white matter hypomyelination in T2WI and FLAIR signal in the periventricular white matter and frontal U-fibers.[sites.google.com]

  • Progressive Bulbar Palsy

    […] paralysis. [68] Spinal muscular atrophy The differential diagnosis of SMA includes congenital muscular dystrophy, congenital myopathy, congenital neuropathy (eg, congenital hypomyelination[emedicine.medscape.com]

  • Myopathy

    Electroencephalographic abnormalities have been noted in several patients with the early-onset form of the disease before clinically apparent seizure activity begins.[anesthesiology.pubs.asahq.org] Congenital hypomyelinating neuropathies. It may be difficult to distinguish myopathy from peripheral neuropathy.[patient.info] […] concentrations usually are normal, although mild elevations are sometimes seen with the early-onset form of the disease. [1] Changes consistent with myopathy are observed on the electromyogram[anesthesiology.pubs.asahq.org]

  • Motor Neuron Disease

    […] paralysis. [68] Spinal muscular atrophy The differential diagnosis of SMA includes congenital muscular dystrophy, congenital myopathy, congenital neuropathy (eg, congenital hypomyelination[emedicine.medscape.com]

  • Diabetic Amyotrophy

    Congenital hypomyelinating neuropathy Congenital hypomyelinating neuropathy. The Schwann cell touches the axon but makes no myelin. There are rings of basement membrane.[neuropathology-web.org] Congenital hypomyelinating neuropathies. It may be difficult to distinguish myopathy from peripheral neuropathy.[patient.info] Hereditary neuropathy with pressure palsies Dejerine-Sottas neuropathy Congenital hypomyelinating neuropathy Roussy-Levy syndrome HSAN HSAN I-Hereditary sensory radicular[neuropathology-web.org]

  • Polyglucosan Body Myopathy Type 2

    4 (HLD4) – Spastic paraplegia 13 Leukodystrophy, hypomyelinating, 3 (HLD3) Limb-girdle muscular dystrophy type 2B Limb-Girdle Muscular Dystrophy type 2C Limb-Girdle Muscular[babygene.co.il] Nerve conduction velocity and electromyogram reveal an axonal lumbosacral polyradiculoneuropathy.[ncbi.nlm.nih.gov] CMT4e (congenital hypomyelinating neuropathy) PMP22, MPZ, ERG-2 g. CMT4f Periaxin 19q13.1-13.3 h. CMT4g 10q23.2 i.[hickoryneurology.net]

  • X-linked Parkinsonism-Spasticity Syndrome

    Edwards Syndrome/Trisomy 18 Fragile X Syndrome Fumarase Deficiency GLUT1-DS Condition Glutaric Aciduria Type 1 Goldenhar’s Syndrome Hunter Syndrome Hurler-Scheie Syndrome Hypomyelination[bladderbowel.gov.au] […] external sphincter electromyogram reflecting loss of anterior horn cells in Onuf's nucleus in the sacral cord is highly suggestive of multiple system atrophy in the appropriate[annalsofian.org] […] spastic paraplegia 17; Neuropathy, distal hereditary motor, type V; Encephalopathy, progressive, with or without lipodystrophy AD, AR 16 HSPD1 118190 spastic paraplegia 13; hypomyelinating[centogene.com]

  • Distal Hereditary Motor Neuropathy Type 5

    DI-CMTB AD 19p12–p13.2 DYN2 DI-CMTC AD 1p34–p35 YARS RI-CMT AR 1p36.31 PLEKHG5 CMT3 (Dejerine–Sottas disease, congenital hypomyelinating neuropathy) AD 17p11.2 PMP22 AD 1q21[mhmedical.com] Skin abnormalities would be heliotrope rash over eyelids, gottrons papules on the knuckles and periungual erythema. [6] Creatinine kinase will be elevated and electromyogram[explainmedicine.com] A – D, MR images of the lumbosacral spine in a patient with the congenital hypomyelinating form of DSD (case 8).[ajnr.org]

  • Hereditary Hyperekplexia

    AR 29 91 POLR3B Leukodystrophy, hypomyelinating AR 19 58 PPT1 * Ceroid lipofuscinosis, neuronal AR 94 77 PRICKLE1 Epilepsy, progressive myoclonic AD/AR 3 16 PRIMA1 Epilepsy[blueprintgenetics.com] […] will not show abnormal activity other than a spike in wakefulness or alertness, while the electromyogram will show rapid muscular responses and hyperreflexia.[en.wikipedia.org] […] or alertness, while the electromyogram will show rapid muscular responses and hyperreflexia.[en.wikipedia.org]

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