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20 Possible Causes for Areflexia, Decreased Number of Myelinated Fibers (Sural Nerve Biopsy)

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  • Déjerine-Sottas Disease

    A sural nerve biopsy specimen showed marked decreases in the numbers of both large and small myelinated fibers, abundant onion-bulb formation, and hypomyelination.[ncbi.nlm.nih.gov] There was a significantly greater incidence of ataxia, areflexia and clinical nerve enlargement in HMSN type III.[ncbi.nlm.nih.gov] […] disease is characterized by an early-onset (i.e. in childhood) demyelinating neuropathy, and usually manifests as gradual progression of distal weakness, sensory loss, and areflexia[radiopaedia.org]

  • Acute Intermittent Porphyria

    Open nerve and muscle biopsies showed significantly decreased numbers of myelinated fibers in the sural nerve and atrophy of type I and II fibers in the quadriceps muscle,[doi.org] The predominantly motor neuropathy associated with weakness and areflexia in AIP can mimic Guillain-Barré syndrome. [7] Nerve conduction studies help in differentiating these[joacp.org] Sensory nerve conduction velocities were absent in ulnar, sural, and plantar nerves, while peroneal motor conduction velocity was decreased (24 m/s; normal, 40 m/s), compatible[doi.org]

  • Xeroderma Pigmentosum

    (D) Representative image of sural nerve biopsy (embedded in Epon and stained with toluidine blue) from patient 1 demonstrating a decrease in the number of myelinated fibers[ng.neurology.org] Neurologic abnormalities were found in 18% of the cases reported, consisting of progressive mental deterioration, hyporeflexia or areflexia, and progressive deafness in some[dx.doi.org]

  • Autonomic Neuropathy

    Pathology Sural nerve biopsy reveals a marked reduction in nerve size and depletion of large and small myelinated fibers but only a slightly decreased number of unmyelinated[ojrd.biomedcentral.com] Neurological studies revealed almost complete sensory loss affecting all modalities in the upper and lower limbs, minimal involvement of motor fibers, and areflexia.[ncbi.nlm.nih.gov] The major urodynamic abnormalities were detrusor areflexia on voiding (5), denervation supersensitivity to bethanechol (3); low compliance detrusor (1); and impaired bladder[ncbi.nlm.nih.gov]

  • Recessive Intermediate Charcot-Marie-Tooth Disease Type D
  • Dysautonomia

    .: Sural nerve biopsy from a CMT1A case.[tankonyvtar.hu] Neurologically Complete Spinal Cord Injury. 38 Darrow D...Samadani U 30667299 2019 15 VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia[malacards.org] Hyporeflexia or areflexia of involved areas represents a major clinical finding on examination of the patient with GBS.[emedicine.medscape.com]

  • Channelopathy-Associated Congenital Insensitivity to Pain

    Pathologically, HSAN II demonstrates a virtual absence of myelinated fibers and a decreased number of unmyelinated fibers in sural nerve biopsies ( table 1 ).[anesthesiology.pubs.asahq.org] The NCS and nerve biopsy are normal. 1 Familial dysautonomia (HSAN-Type-III) also comprises of nystagmus, hypotonia, areflexia and poor coordination. 6 Asymbolia for pain[apicareonline.com] Pathology Sural nerve biopsy reveals a marked reduction in nerve size and depletion of large and small myelinated fibers but only a slightly decreased number of unmyelinated[ojrd.biomedcentral.com]

  • Autosomal Dominant Hereditary Sensory and Autonomic Neuropathy

    Pathologically, HSAN II demonstrates a virtual absence of myelinated fibers and a decreased number of unmyelinated fibers in sural nerve biopsies ( table 1 ).[anesthesiology.pubs.asahq.org] The weakness develops in 2-4 weeks, proximal and upper limb dominant, followed by areflexia and moderate sensory disturbances.[tankonyvtar.hu] […] mottling. [10] Holmes-Adie syndrome and Ross syndrome: Holmes-Adie syndrome is probably autoimmune in nature and manifests as tonic pupil or pupils associated with tendon areflexia[emedicine.medscape.com]

  • Hereditary Sensory Neuropathy

    Pathology Sural nerve biopsy reveals a marked reduction in nerve size and depletion of large and small myelinated fibers but only a slightly decreased number of unmyelinated[doi.org] […] mottling. [10] Holmes-Adie syndrome and Ross syndrome: Holmes-Adie syndrome is probably autoimmune in nature and manifests as tonic pupil or pupils associated with tendon areflexia[emedicine.com] Although the tendon reflexes are decreased or absent, there is no muscle atrophy or muscle weakness.[doi.org]

  • Familial Dysautonomia

    Pathology Sural nerve biopsy reveals a marked reduction in nerve size and depletion of large and small myelinated fibers but only a slightly decreased number of unmyelinated[doi.org] An infant presented with congenital hypotonia, poor sucking, apathy, and areflexia.[ncbi.nlm.nih.gov] Autonomic abnormalities include labile cardiovascular function, lack of corneal reflexes leading to corneal scarring, areflexia, and absent axonal flare response after intradermal[ncbi.nlm.nih.gov]

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