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15 Possible Causes for Decerebrate Posturing, Optic Disc Pallor, Spastic Dystonia

  • Globoid Cell Leukodystrophy

    Eventually, they present decerebrate posturing and die. Late-onset GLC is also characterized by normal development until symptom onset.[symptoma.com] Signs included pes cavus, optic disc pallor, progressive spastic tetraparesis, a sensorimotor demyelinating neuropathy and hypodense lesions in the parieto-occipital periventricular[ncbi.nlm.nih.gov] , dystonia, ataxia and peripheral neuropathy.[ncbi.nlm.nih.gov]

  • Subacute Sclerosing Panencephalitis

    There was evidence of decerebrate posturing.[ncbi.nlm.nih.gov] Right fundus showed mild disc pallor with full cup and blurred margins suggestive of papillitis developing secondary optic atrophy [Figure - 1].[ijo.in] The visual problems and myoclonic jerks are typical for stage II, and stage III symptoms include dystonias, choreoathetosis and spasticity.[path.upmc.edu]

  • Spastic Ataxia with Congenital Miosis

    2: extensor (rigid) response, decerebrate posture 1: none Term Definition Hydrocephalus: Presenting sxs are related to the age of the patient: - young, nonverbal patient[flashcardmachine.com] disc pallor Sensory axonal neuropathy Flexion contracture Limb muscle weakness Progressive spastic paraplegia Scoliosis Motor axonal neuropathy Violent behavior Inertia Exaggerated[mendelian.co] , Angelman syndrome , Ataxia spastic congenital miosis , Ataxia , Athetosis , Autonomic dysreflexia , Autonomic dystonia , Autosomal recessive spastic paraplegia , Bahemuka[wikidoc.org]

  • Autosomal Recessive Isolated Optic Atrophy

    posturing), but the pace slows down, and symptoms may stabilize for one to two years.[neuroweb.us] The families described by Hanein et al. [ 4 ] were characterized by early-onset severe visual impairment, optic disc pallor, and central scotomata.[molvis.org] Diagnosis is mainly clinical based on history, clinical examination, and fundoscopic findings of pallor/edema of the optic disc and peripapillary hemorrhage.[ijri.org]

  • Mitochondrial Disease

    Following control of seizures the child went into decerebrate posturing. The raised intracranial tension was treated with Mannitol (i.v., 5 mg stat).[ijponline.biomedcentral.com] Fundoscopic examination reveals temporal pallor of the optic disc.[neuropathology-web.org] A broad spectrum of movement disorders can manifest in mitochondrial diseases including ataxia, Parkinsonism, myoclonus, dystonia, choreoathetosis, spasticity, tremor, tic[ncbi.nlm.nih.gov]

  • Lethal Ataxia with Deafness and Optic Atrophy

    posturing can be seen as well as blindness CSF: increased protein, sulfatide in urine MRI: symmetric demyelination SPARING SUBCORTICAL U-FIBERS NCS: slowed motor and sensory[quizlet.com] Examination of these patients shows loss of visual acuity, temporal pallor of the optic discs, centrocecal scotomas with peripheral sparing, and subtle impairments in color[en.wikipedia.org] (late) • Others… – optic atrophy, pigment degeneration, nystagmus – ataxia (if able to stand) – dystonia , multifocal myoclonus – seizures Pathological Features • bilateral[emilytam.com]

  • Early-Onset X-Linked Isolated Optic Atrophy

    posturing), but the pace slows down, and symptoms may stabilize for one to two years.[neuroweb.us] The families described by Hanein et al. [ 4 ] were characterized by early-onset severe visual impairment, optic disc pallor, and central scotomata.[molvis.org] For example, 1-the syndrome of LHON plus spastic dystonia and basal ganglionic lesions has been reported in at least two 11778 families and in one 3460 family. 2-the syndrome[slideplayer.com]

  • Spastic Paraplegia - Ataxia - Mental Retardation

    posturing), but the pace slows down, and symptoms may stabilize for one to two years.[neuroweb.us] disc pallor Brain atrophy Abnormality of eye movement Clumsiness Intellectual disability, severe Progressive encephalopathy Muscular dystrophy Cerebral dysmyelination Perisylvian[mendelian.co] Abstract We reported three siblings with complicated hereditary spastic paraplegia.[ncbi.nlm.nih.gov]

  • Autosomal Recessive Non-Syndromic Hydrocephalus

    Leukodystrophy Leukoencephalopathy Horizontal nystagmus Frequent falls Tetraparesis Hyperactive deep tendon reflexes Autoimmune thrombocytopenia Abnormality of movement Decerebrate[mendelian.co] Color fundus imaging ( A, C, F, I ) in four patients demonstrated optic disc pallor, attenuated vessels, macular and midperipheral retinal pigment epithelium mottling/atrophy[iovs.arvojournals.org] […] paraplegia 7, autosomal recessive 607259 602783 Autosomal dominant; Autosomal recessive SPR 2p13.2 Dystonia, dopa-responsive, due to sepiapterin reductase deficiency 612716[mnglabs.com]

  • Spinocerebellar Ataxia with Rigidity and Peripheral Neuropathy

    D Symptoms Decerebrate posture Decerebrate posture is an abnormal body posture that involves the arms and legs being held straight out, the toes being pointed downward, and[mountsinai.org] disc pallor Distal lower limb muscle weakness Generalized limb muscle atrophy Stuttering Dysphagia Difficulty walking Postural instability Bradykinesia Torticollis Acute[mendelian.co] […] ataxias Extrapyramidal Akinesia/rigidity/dystonia: SCA 3 , 9 , 17 , 21 ; 12 (Akinesia) Chorea Early/prominent: DRPLA , 17 , 27 ; Rarely SCA 2 Dyskinesia: SCA 27 Myoclonus[neuromuscular.wustl.edu]

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