Research Topics
Species | A F HahnSummaryAffiliation: University of Western Ontario Country: Canada Publications
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Publications
Cold-induced sweating syndrome: CISS1 and CISS2: manifestations from infancy to adulthood. Four new casesA F Hahn
Department of Clinical Neurological Sciences, London Health Sciences Centre, University of Western Ontario, London, Ontario, Canada
J Neurol Sci 293:68-75. 2010..Detailed clinical observations, correlated with the findings from basic science research, may serve to elucidate the role(s) of this important cytokine complex in embryonic and postnatal development...
Cold-induced sweating syndrome: a report of two cases and demonstration of genetic heterogeneityA F Hahn
Department of Clinical Neurological Sciences, London Health Science Center, University of Western Ontario, London, Canada N6A 5A5
J Neurol Sci 250:62-70. 2006..This is the first detailed report on the clinical consequences of mutations in the CLCF1 gene in humans. Directions for medical therapies are outlined to achieve long term symptom control...
Pathological findings in the x-linked form of Charcot-Marie-Tooth disease: a morphometric and ultrastructural analysisA F Hahn
Department of Clinical Neurological Sciences, The University of Western Ontario, London Health Sciences Centre, Canada
Acta Neuropathol 101:129-39. 2001..We conclude that mutations in Cx 32 gap junction protein lead to a compromise of Schwann cell functions and to impaired Schwann cell-axon interactions with subsequent pathology in both myelin and axons...
Clinical and pathological observations in men lacking the gap junction protein connexin 32A F Hahn
Department of Clinical Neurological Sciences, London Health Sciences Centre, University of Western Ontario, London, Canada
Muscle Nerve Suppl 9:S39-48. 2000..The predicted absence of Cx 32 gap junctions is shown to be associated with a severe dysfunction of the axon-Schwann cell unit. Observed changes resemble those of Cx 32-null mice. No central nervous system changes were demonstrated...
Intravenous immunoglobulin treatment in peripheral nerve disorders--indications, mechanisms of action and side-effectsA F Hahn
London Health Sciences Centre, The University of Western Ontario, Canada
Curr Opin Neurol 13:575-82. 2000..New knowledge that advances the understanding of the pathogenesis of the neuropathies and of the mechanisms of action of IVIg is discussed...
Guillain-Barré syndromeA F Hahn
Clinical Neurological Sciences, University of Western Ontario, London Health Sciences Centre, Canada
Lancet 352:635-41. 1998..Admission to the intensive-care unit is necessary in 33% of patients who require intubation and assisted ventilation. Immunomodulation with infusions of IgG or plasma exchange treatments foreshorten the disease course...
P2-peptide induced experimental allergic neuritis: a model to study axonal degenerationA F Hahn
Department of Clinical Neurological Sciences, University of Western Ontario, Victoria Hospital, London, Canada
Acta Neuropathol 82:60-5. 1991..Low doses resulted in pure demyelination. Axonal degeneration occurred only with high doses of antigen and in association with very active mononuclear inflammation. The role of macrophages in producing axonal damage is discussed...
Antigalactocerebroside antibody increases demyelination in adoptive transfer experimental allergic neuritisA F Hahn
Department of Clinical Neurological Sciences, University of Western Ontario, Victoria Hospital, London, Canada
Muscle Nerve 16:1174-80. 1993..The observations support the concept of a synergistic role of T-cell autoimmunity and humoral responses in the inflammatory demyelination of Lewis rat EAN...
X-linked dominant hereditary motor and sensory neuropathyA F Hahn
Department of Clinical Neurological Sciences, University of Western Ontario, Victoria Hospital, London, Canada
Brain 113:1511-25. 1990..Nerve biopsies showed loss of myelinated and unmyelinated nerve fibres, regenerative sprouting and secondary demyelination. The findings indicate that this distinct variant of HMSN is the result of primary axonal degeneration...
Genotype/phenotype correlation in affected individuals of a family with a deletion of the entire coding sequence of the connexin 32 geneP J Ainsworth
Molecular Diagnostic Laboratory, London Health Sciences Centre, Ontario, Canada
Hum Genet 103:242-4. 1998..Studies of kindreds such as this, where in CMTX-affected males the Cx32 gene product is totally absent, will help us to better understand the molecular mechanisms underlying the clinical phenotype associated with this disorder...
Novel mutations in the connexin 32 gene associated with X-linked Charcot-Marie tooth diseaseC C Tan
Department of Medical Biochemistry, University of Western Ontario, Victoria Hospital, London, Canada
Hum Mutat 7:167-71. 1996
Neuromyotonia in hereditary motor neuropathyA F Hahn
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada
J Neurol Neurosurg Psychiatry 54:230-5. 1991..Symptoms were improved with diphenylhydantoin, carbamazepine and tocainide...
Rigid spine muscular dystrophy due to SEPN1 mutation presenting as cor pulmonaleS L Venance
London Health Sciences Center, University of Western Ontario, London, ON, Canada
Neurology 64:395-6. 2005
Adoptive transfer of experimental allergic neuritis in the immune suppressed hostA F Hahn
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada
Acta Neuropathol 86:596-601. 1993..Irradiated recipients developed similar clinical EAN and showed macrophage-mediated demyelination despite severe suppression of the host inflammatory response...
A novel sodium channel mutation in a family with hypokalemic periodic paralysisD E Bulman
Division of Neurology, Ottawa General Hospital, Ottawa Hospital Research Institute, Ontario, Canada
Neurology 53:1932-6. 1999..To identify the cause of hypokalemic periodic paralysis (HOKPP) in a family whose disease is not caused by a mutation in the dihydropyridine-sensitive (DHP) receptor alpha1-subunit gene (CACNA1S)...
Reliability and validity of the CMT neuropathy score as a measure of disabilityM E Shy
Department of Neurology, Wayne State University, Detroit, MI 48201, USA
Neurology 64:1209-14. 2005..To determine the validity and reliability of the Charcot-Marie-Tooth disease (CMT) neuropathy score (CMTNS) in patients with inherited neuropathy...
