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Sensory Axonal Neuropathy (sensory axonal + neuropathy)

Distribution by Scientific Domains

Medical Sciences	80%

Selected Abstracts

CLINICAL, MRI, AND SKIN BIOPSY FINDINGS IN SENSORY GANGLIONOPATHIES

JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000
A. Sghirlanzoni
Unlike peripheral motor disorders, sensory disturbances are rarely diagnosed by the probable site of pathology. This approach is useful in the differential diagnosis between chronic sensory axonal neuropathies and ganglionopathies, in which routine clinical and neurophysiological evaluation alone often do not provide definite clues. Methods: Thirty patients with peripheral sensory disturbances were investigated. MRI was performed at cervical level in all cases. Four patients also underwent thoracic and lumbar MRI. Seventeen patients underwent skin biopsy at the proximal thigh and the distal leg. In 4 of them, further skin biopsies were taken at C5 dermatome and at the hand. Density of intra-epidermal nerve fibers (IENF) was quantified. Results: In 22 patients, sensory ganglionopathy was suspected. Disease was idiopathic in 7 cases; paraneoplastic in 3 cases; and associated with Sjögren, AIDS, autoimmune chronic hepatitis, and cisplatin neurotoxicity in 4 cases. One patient had a hereditary sensory autonomic neuropathy. Four patients had vitamin E deficiency and 3 patients a spinocerebellar syndrome. In 8 patients, sensory axonal neuropathy related to diabetes, alcoholism, and AIDS on antiretroviral treatment, and monoclonal gammopathy of undetermined significance was diagnosed. MRI findings: All ganglionopathy patients showed posterior columns hyperintensity on T2-weighted MRI. Conversely, MRI was negative in all axonal sensory neuropathy patients. Skin biopsy findings: In neuropathies, IENF density was significantly lower at the distal leg than at the proximal thigh, while ganglionopathies did not show any change with respect to the rostral:caudal orientation. A similar pattern of epidermal denervation was observed in the arm. Discussion: The degeneration of both central and peripheral sensory pathway in a fashion that is not length-dependent localizes the disease to T-shaped sensory neurons Early ataxia and cutaneous sensory symptoms involving the proximal regions of the body reflect this pattern of denervation and should prompt the diagnosis of ganglionopathy. This can be confirmed by T2-weighted hyperintensity in the posterior columns and a distinct pattern of IENF loss. [source]

Electrophysiological sensory demyelination in typical chronic inflammatory demyelinating polyneuropathy

EUROPEAN JOURNAL OF NEUROLOGY, Issue 7 2010
Y. A. Rajabally
Background:, The presence of electrophysiological demyelination of sensory nerves is not routinely assessed in the evaluation of suspected chronic inflammatory demyelinating polyneuropathy (CIDP). Whether this can be useful is unknown. Methods:, We compared, using surface recording techniques, in 19 patients with typical CIDP and 26 controls with distal large fibre sensory axonal neuropathy, the forearm median sensory conductions, sensory nerve action potential (SNAP) amplitudes and durations and sensory nerve conduction velocities (SNCVs) of median, radial and sural nerves. Results:, Median nerve sensory conduction block (SCB) across the forearm was greater in CIDP patients than in controls (P = 0.005). SNAP durations were longer in CIDP patients for median (P = 0.001) and sural nerves (P = 0.004). Receiver operating characteristic (ROC) curves provided sensitive (>40%) and specific (>95%) cut-offs for median nerve SCB as well as median and sural SNAP durations. SNCVs were significantly slower for median and sural nerves in CIDP patients, but ROC curves did not demonstrate cut-offs with useful sensitivities/specificities. Median SCB or prolonged median SNAP duration or prolonged sural SNAP duration offered a sensitivity of 73.7% for CIDP and specificity of 96.2%. Used as additional parameters, they improved diagnostic sensitivity of the American Academy of Neurology (AAN) criteria for CIDP of 1991, from 42.1% to 78.9% in this population, with preserved specificity of 100%. Discussion:, Sensory electrophysiological demyelination is present and may be diagnostically useful in typical CIDP. SCB detection and SNAP duration prolongation appear to represent more useful markers of demyelination than SNCV reduction. [source]

Clinical presentation and prognosis of childhood Guillain,Barré syndrome

JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 7-8 2008
Jung Hwan Lee
Aim: Guillain,Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterised by rapidly progressive, symmetric weakness and areflexia. This study is to assess the clinical characteristics of paediatric GBS, as well as its long-term functional prognosis. Methods: We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 56 children diagnosed with GBS. Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy ([AIDP]n = 34), acute motor axonal neuropathy ([AMAN]n = 14), acute motor and sensory axonal neuropathy (n = 1) and Miller Fisher syndrome ([MFS]n = 7). Results: Upper respiratory infection was the most frequent preceding event, and limb weakness was the most frequent symptom at GBS onset. There was no significant difference in the mean time from the onset of illness to nadir between any of these groups. Both the AIDP and AMAN groups showed significantly poorer functional status, measured by the Hughes scale, than the MFS group. Two years after nadir, however, the three groups did not differ significantly. Functional status at nadir, as estimated by the Hughes scale, is a more important factor than electrophysiological types in predicting long-term outcome. Conclusion: The most common symptom at onset in paediatric GBS was limb weakness. Functional status at nadir in AMAN was not significantly different from that of AIDP, and both types achieved good functional outcome for ambulation after 2 years. Functional status at nadir was more important than the electrophysiological type in predicting long-term outcomes. [source]

CLINICAL, MRI, AND SKIN BIOPSY FINDINGS IN SENSORY GANGLIONOPATHIES

Spastic Paraplegia, Optic Atrophy, and Neuropathy: New Observations, Locus Refinement, and Exclusion of Candidate Genes

ANNALS OF HUMAN GENETICS, Issue 3 2009
Lúcia Inês Macedo-Souza
Summary SPOAN is an autosomal recessive neurodegenerative disorder which was recently characterized by our group in a large inbred Brazilian family with 25 affected individuals. This condition is clinically defined by: 1. congenital optic atrophy; 2. progressive spastic paraplegia with onset in infancy; and 3. progressive motor and sensory axonal neuropathy. Overall, we are now aware of 68 SPOAN patients (45 females and 23 males, with age ranging from 5 to 72 years), 44 of which are presented here for the first time. They were all born in the same geographic micro region. Those 68 patients belong to 43 sibships, 40 of which exhibit parental consanguinity. Sixty-one patients were fully clinically evaluated and 64 were included in the genetic investigation. All molecularly studied patients are homozygotes for D11S1889 at 11q13. This enabled us to reduce the critical region for the SPOAN gene from 4.8 to 2.3 Mb, with a maximum two point lod score of 33.2 (with marker D11S987) and of 27.0 (with marker D11S1889). Three genes located in this newly defined critical region were sequenced, but no pathogenic mutation was detected. The gene responsible for SPOAN remains elusive. [source]