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106 Possible Causes for Ataxia, Onset of Symptoms in First or Second Decade of Life

  • Hallervorden-Spatz Syndrome

    There is an early onset classic form with symptoms of extrapyramidal disease beginning in the first decade of life and rapid progression to loss of ambulation in about 15[] The patient presented with a slowly progressive illness with seizures, extrapyramidal symptoms, cerebellar ataxia, dementia, spasticity, myoclonic movements and a severe demyelinating[] Although rare, cerebellar ataxia, behavioural abnormalities, parkinsonism and apraxia of eyelid opening were exclusively seen in late onset patients.[]

  • Huntington's Disease

    However, in some families a juvenile form of HD has been seen with an onset of symptoms in the first or second decades of life.[] We screened 89 patients with a Huntington's disease-like phenotype without the HD-gene mutation and 178 patients with genetically unclassified cerebellar ataxia for the mutation[] The authors highlight the importance of suspecting HD in the aetiology of spinocerebellar ataxias when dementia is a prominent feature in the proband or their family.[]

  • Hereditary Areflexic Dystasia

    Age of onset is in the second and third decades of life and rarely in the first. Recovery is usually complete and begins weeks to months after the onset of symptoms.[] Cerebellar ataxia, early-onset Cerebellar ataxia, late-onset Drug-induced cerebellar ataxia Early onset cerebellar ataxia Early onset cerebellar ataxia with essential tremor[] 2012 ICD-9-CM Diagnosis Code 334.3 Other cerebellar ataxia Short description: Cerebellar ataxia NEC.[]

  • Hypertrophic Interstitial Neuropathy

    […] of symptoms usually in the second decade Progression is slower than in type 1 Nerve conduction studies are usually normal, but electromyography shows evidence of denervation[] Synonym(s): interstitial hypertrophic neuropathy Roussy-Lévy disease - a type of cerebellar ataxia regularly associated with wasting of the calves and intrinsic muscles of[] , ataxy, ataxic R27.0 ICD-10-CM Diagnosis Code R27.0 Ataxia, unspecified 2016 2017 2018 2019 Billable/Specific Code Type 1 Excludes ataxia following cerebrovascular disease[]

  • Friedreich Ataxia

    Most affected people become wheelchair dependent by the second or third decade of life.[] Early onset cerebellar ataxia with retained tendon reflexes: a clinical and genetic study of a disorder distinct from Friedreich's ataxia.[] Convert to ICD-10-CM : 334.0 converts approximately to: 2015/16 ICD-10-CM G11.1 Early-onset cerebellar ataxia Approximate Synonyms Cervical neuromuscular scoliosis due to[]

  • Hereditary Angioedema

    Onset of clinical symptoms occurred in the first decade of life in 11 (8%) patients, in the second decade in 60 (43.5%) patients, in the third decade in 22 (15.9%) patients[] Episodes of severe headaches, visual disturbances (eg, blurred vision, diplopia), and ataxia have been reported.[] Onset of clinical symptoms In a series of 138 patients, the mean age at onset of the disease was 26.8 years (SD /- 14.9 years, range 1 to 68 years) [ 12 ].[]

  • Hereditary Spastic Paraplegia

    Clinical Correlations: Spastic paraplegia 7 may have its onset in the second decade of life but sometimes much later.[] INTRODUCTION: The combination of cerebellar ataxia and spasticity is common.[] Criteria for hereditary ataxia The following groups were included: subjects with progressive cerebellar ataxia and positive family history or confirmed genetic diagnosis;[]

  • 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.[] We review the differential diagnosis of cerebellar ataxia with vision loss secondary to retinal pigmentary dystrophy.[] In addition to the main clinical symptoms, at least one of the following is also often present: heart block, cerebellar ataxia, or cerebrospinal fluid (CSF) protein content[]

  • AMACR Deficiency

    Similarly, the single patient with sterol carrier protein X (SCPX, now named SCP2) deficiency first experienced neurologic symptoms in the second decade of life ( Ferdinandusse[] AMACR mutations cause late-onset autosomal recessive cerebellar ataxia. Neurology. 2011 May 17;76(20):1768-70. doi: 10.1212/WNL.0b013e31821a4484.[] Symptoms associated with the central or peripheral nervous system predominate and generally include encephalopathy, hearing and visual impairment or complete loss, epilepsy and cerebellar[]

  • Spastic Paraplegia

    Clinical Correlations: Spastic paraplegia 7 may have its onset in the second decade of life but sometimes much later.[] Palau F, Espinós C: Autosomal recessive cerebellar ataxias.[] Abstract Hereditary ataxia and spastic paraplegia are heterogeneous monogenic neurodegenerative disorders.[]

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