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28 Possible Causes for Hyperreflexia, Neuronal Loss and Gliosis in the Inferior Olives

  • Severe Neonatal-Onset Encephalopathy with Microcephaly

    Hyperreflexia MedGen UID: 57738 • Concept ID: C0151889 • Finding Autonomic nervous system overreaction to stimuli, most commonly after spinal cord injury at a T-5 level and[ncbi.nlm.nih.gov] Postmortem examination of the brain in one child disclosed severe neuronal loss in the inferior olives and the pontine nuclei.[link.springer.com] Spasticity and hyperreflexia are often present but some newborn infants are hypotonic.[disorders.eyes.arizona.edu]

  • Spinocerebellar Ataxia Type 13

    : Clinical features Age of onset - 43-56 years Slow progression Gait and limb ataxia Dysarthria Occular dysmetria Slow saccades Decreased vibratory sense below the knees Hyperreflexia[emedicine.com] loss and gliosis.[link.springer.com] Affected individuals show mild to profound mental retardation with poor or absent speech as well as gait and stance ataxia and hyperreflexia.[uniprot.org]

  • Adult-Onset Autosomal Recessive Cerebellar Ataxia

    : Clinical features Age of onset - 43-56 years Slow progression Gait and limb ataxia Dysarthria Occular dysmetria Slow saccades Decreased vibratory sense below the knees Hyperreflexia[emedicine.medscape.com] Microscopic examination of these structures reveals neuronal loss, axon and myelin degeneration, and gliosis.[neuropathology-web.org] […] and decreased ankle reflexes); movement disorders such as chorea, dystonia, and oculomotor abnormalities; pyramidal tract dysfunction such as extensor plantar responses, hyperreflexia[blogs.nejm.org]

  • Spinocerebellar Ataxia Type 5

    All had at least mild hyperreflexia, with evidence of abnormal reflex spread in the upper and/or lower limbs in four of the six subjects.[jnnp.bmj.com] loss and gliosis.[link.springer.com] […] ataxia type 7 (SCA 7) is a polyglutamine (polyQ) neurodegenerative disease characterized by progressive cerebellar ataxia and retinal degeneration with ophthalmoplegia, hyperreflexia[oatext.com]

  • Autosomal Recessive Spinocerebellar Ataxia

    : Clinical features Age of onset - 43-56 years Slow progression Gait and limb ataxia Dysarthria Occular dysmetria Slow saccades Decreased vibratory sense below the knees Hyperreflexia[emedicine.medscape.com] Microscopic examination of these structures reveals neuronal loss, axon and myelin degeneration, and gliosis.[neuropathology-web.org] Typical clinical signs and symptoms of autosomal dominant ataxias (ADCA) include the following: Limb and truncal ataxia Hyperreflexia and spasticity (pyramidal signs) are[centogene.com]

  • Friedreich Ataxia

    We report two patients with delayed-onset, hyperreflexia and gradually progressive disease.[ncbi.nlm.nih.gov] Microscopic examination of these structures reveals neuronal loss, axon and myelin degeneration, and gliosis.[neuropathology-web.org] His examination was significant for spastic gait, hyperreflexia, and sensory neuropathy. Genetic testing revealed a compound heterozygous mutation in the FXN gene.[ncbi.nlm.nih.gov]

  • Fatal Familial Insomnia

    Neuronal loss and gliosis are most conspicuous and frequent in inferior olives and minor in other nuclei ( Manetto et al ., 1992 ; Gambetti et al ., 1995 ; Reder et al .,[brain.oxfordjournals.org]

  • Striatonigral Degeneration

    In PD, urgency to void, with or without difficulty voiding, but without chronic retention, associated with detrusor hyperreflexia and normal urethral sphincter function, predominated[ncbi.nlm.nih.gov] There is neuronal loss and gliosis in the inferior olives, pons, cerebellum, substantia nigra, locus ceruleus, striatum and the intermediolateral column of the spinal cord[dizziness-and-balance.com] Other symptoms can include depression, emotional lability (fluctuations of emotional state), hyperreflexia, extensor plantar (sole) response, myoclonus, or laryngeal stridor[encyclopedia.com]

  • Shy Drager Syndrome

    Urodynamic studies revealed detrusor areflexia in 6 patients (67 per cent), detrusor hyperreflexia in 3 (33 per cent), a lower motor neuron lesion involving the periurethral[ncbi.nlm.nih.gov] loss, gliosis, and microvacuolation in the involved neuronal systems.[emedicine.medscape.com] (Corticospinal tract dysfunction with extensor plantar response with hyperreflexia [pyramidal sign] is not used to categorize MSA.) (See DDx.) Table 2a.[emedicine.com]

  • Autosomal Recessive Spinocerebellar Ataxia 8

    : Clinical features Age of onset - 43-56 years Slow progression Gait and limb ataxia Dysarthria Occular dysmetria Slow saccades Decreased vibratory sense below the knees Hyperreflexia[emedicine.medscape.com] Microscopic examination of these structures reveals neuronal loss, axon and myelin degeneration, and gliosis.[neuropathology-web.org] Cerebellar atrophy Degeneration of cerebellum 0001272 Cerebellar vermis atrophy 0006855 Dystonia 0001332 Gait ataxia Inability to coordinate movements when walking 0002066 Hyperreflexia[rarediseases.info.nih.gov]

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