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12 Possible Causes for Bilateral Babinski's Reflex, Onset of Symptoms in First or Second Decade of Life, Psychomotor Retardation

  • Tay-Sachs Disease

    Late-onset Tay-Sachs disease (chronic form) is a rare variant phenotype with appearance of first symptoms during the second or third decade of life. 4 A juvenile form is also[doi.org] The patients was a 3-year-old boy with psychomotor retardation and attacked by seizures since 8 months of age. On funduscopy, the maculla presented a cherry-red spot.[ncbi.nlm.nih.gov] Psychomotor retardation appears after the age of 8 months with hypotonia, amaurosis, and megalencephaly. A cherry-red macular spot may be found but is not specific.[orpha.net]

  • Spastic Paraplegia

    Clinical Correlations: Spastic paraplegia 7 may have its onset in the second decade of life but sometimes much later.[disorders.eyes.arizona.edu] On neurological examination, he showed hyperreflexia, spasticity, and weakness in the lower extremities and bilateral Babinski reflexes.[ncbi.nlm.nih.gov] Retardation and Characteristic Facies 2 AR 616801 Mast Syndrome AR 248900 Mental Retardation, X-Linked, Syndromic, Claes-Jensen Type XL 300534 Neuropathy, Hereditary Sensory[preventiongenetics.com]

  • Paraplegia

    Clinical Correlations: Spastic paraplegia 7 may have its onset in the second decade of life but sometimes much later.[disorders.eyes.arizona.edu] There was hyperreflexia on bilateral deep tendon reflex testing, and Babinski sign was positive bilaterally.[j-neurooncology.imedpub.com] retardation with or without seizures AR 13 13 HSPD1 * Spastic paraplegia, Leukodystrophy, hypomyelinating AD/AR 5 5 IBA57 Multiple mitochondrial dysfunctions syndrome 3,[blueprintgenetics.com]

  • Friedreich Ataxia

    Most affected people become wheelchair dependent by the second or third decade of life.[web.archive.org] His reflexes were absent and Babinski sign was present bilaterally. A nine-year-old sister was found to have mild ataxia but was otherwise neurologically intact.[ncbi.nlm.nih.gov] […] and dysequlibrium syndrome CEREBELLAR ATROPHY, DEVELOPMENTAL DELAY, AND SEIZURES Cerebellar Atrophy, Visual Impairment, and Psychomotor Retardation Charlevoix-Saguenay spastic[rgd.mcw.edu]

  • Kearns-Sayre Syndrome

    Signs and symptoms [ edit ] Individuals with KSS present initially in a similar way to those with typical CPEO. Onset is in the first and second decades of life.[en.wikipedia.org] View Article PubMed Google Scholar Ramaekers VT, Hausler M, Opladen T, Heimann G, Blau N: Psychomotor retardation, spastic paraplegia, cerebellar ataxia and dyskinesia associated[ncbi.nlm.nih.gov] retardation, parkinsonism, gait difficulties, sensory ataxia Cognitive decline, cerebral atrophy, peripheral neuropathy Endocrinopathies (diabetes, infertility) Mood disorders[ncbi.nlm.nih.gov]

  • Hereditary Spastic Paraplegia

    Clinical Correlations: Spastic paraplegia 7 may have its onset in the second decade of life but sometimes much later.[disorders.eyes.arizona.edu] Retardation and Characteristic Facies 2 AR 616801 Mast Syndrome AR 248900 Mental Retardation, X-Linked, Syndromic, Claes-Jensen Type XL 300534 Neuropathy, Hereditary Sensory[preventiongenetics.com] retardation with or without seizures AR 10 11 HSPD1 * Spastic paraplegia, Leukodystrophy, hypomyelinating AD/AR 4 4 IBA57 Multiple mitochondrial dysfunctions syndrome 3,[blueprintgenetics.com]

  • Autosomal Dominant Spastic Paraplegia Type 42

    onset, usually within the first or second decades of life.[jamanetwork.com] Deep tendon reflexes were increased in all patients with clonus in 3 and bilateral Babinski sign in 4.[bmcneurol.biomedcentral.com] CCHLND is an autosomal recessive disorder characterized by congenital cataracts, severe psychomotor retardation, and hearing loss associated with decreased serum ceruloplasmin[slc.bioparadigms.org]

  • Autosomal Dominant Spastic Paraplegia Type 10

    onset, usually within the first or second decades of life.[jamanetwork.com] Deep tendon reflexes were increased in all patients with clonus in 3 and bilateral Babinski sign in 4.[bmcneurol.biomedcentral.com] retardation with or without seizures AR 13 13 HSPD1 * Spastic paraplegia, Leukodystrophy, hypomyelinating AD/AR 5 5 IBA57 Multiple mitochondrial dysfunctions syndrome 3,[blueprintgenetics.com]

  • Autosomal Recessive Spastic Paraplegia Type 7

    Clinical Correlations: Spastic paraplegia 7 may have its onset in the second decade of life but sometimes much later.[disorders.eyes.arizona.edu] The deep tendon reflexes were increased in all four limbs, but Babinski sign was not elicited bilaterally. Neither ankle nor knee clonus was observed.[nature.com] retardation; the late childhood form causes poor coordination, gait and language acquisition disorders, and gelastic cataplexy; the juvenile form causes ataxia, epilepsy,[elsevier.es]

  • Lethal Muscular Hypertonia

    The average age at which symptoms first appear is 28, but onset can occur anywhere from the second to the fifth decades of life.[ulf.org] Exaggerated deep-tendon reflexes and Babinski reflex were also observed. Irritabilities and myoclonus of the limbs were readily induced by stimulation.[nature.com] The other one had brachycephaly, and psychomotor retardation.[tmj.ro]

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