Literature DB >> 3003009

Porphyric neuropathy: a clinical, neurophysiological and morphological study.

C A Defanti, A Sghirlanzoni, E Bottacchi, D Peluchetti.   

Abstract

A case of neuropathy in the course of an attack of acute intermittent porphyria was studied from the neurophysiological and morphological points of view. The neurophysiological findings (acute neuropathy with almost complete denervation despite normal or slightly reduced conduction velocity) and the morphological findings (no segmental demyelination after teasing, conservation of the linear fiber diameter/internodal distance ratio, mainly axonal damage on ultrastructural study) seem to indicate that the disease process is chiefly an axonal neuropathy.

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Year:  1985        PMID: 3003009     DOI: 10.1007/bf02331049

Source DB:  PubMed          Journal:  Ital J Neurol Sci        ISSN: 0392-0461


  12 in total

1.  ON THE NATURE OF THE PERIPHERAL NERVE LESIONS ASSOCIATED WITH ACUTE INTERMITTENT PORPHYRIA.

Authors:  J B CAVANAGH; R S MELLICK
Journal:  J Neurol Neurosurg Psychiatry       Date:  1965-08       Impact factor: 10.154

2.  The neuropathology of acute porphyria.

Authors:  J B GIBSON; A GOLDBERG
Journal:  J Pathol Bacteriol       Date:  1956-04

3.  Peripheral nerve changes in porphyric neuropathy: findings in a sural nerve biopsy.

Authors:  A P Anzil; S Dozić
Journal:  Acta Neuropathol       Date:  1978-05-24       Impact factor: 17.088

4.  delta-Aminolevulinic acid: influences on synaptic GABA receptor binding may explain CNS symptoms of porphyria.

Authors:  W E Müller; S H Snyder
Journal:  Ann Neurol       Date:  1977-10       Impact factor: 10.422

5.  Acute intermittent porphyria. Increased ALA-synthetase activity during an acute attack.

Authors:  V P Sweeney; M A Pathak; A K Asbury
Journal:  Brain       Date:  1970       Impact factor: 13.501

6.  The normal sural nerve in man. II. Changes in the axons and Schwann cells due to ageing.

Authors:  J Ochoa; W G Mair
Journal:  Acta Neuropathol       Date:  1969       Impact factor: 17.088

7.  The normal sural nerve in man. I. Ultrastructure and numbers of fibres and cells.

Authors:  J Ochoa; W G Mair
Journal:  Acta Neuropathol       Date:  1969       Impact factor: 17.088

8.  Delta amino levulinic acid. Effect of a porphyrin precursor on an isolated neuronal preparation.

Authors:  H N Dichter; L Taddeini; S Lin; G F Ayala
Journal:  Brain Res       Date:  1977-04-22       Impact factor: 3.252

9.  Porphyric polyneuropathy and its pathogenesis in the light of electrophysiological investigations.

Authors:  D Wochnik-Dyjas; M Niewiadomska; E Kostrzewska
Journal:  J Neurol Sci       Date:  1978-02       Impact factor: 3.181

10.  Electromyogram and nerve conduction in patients with acute intermittent porphyria.

Authors:  K A Flügel; K F Druschky
Journal:  J Neurol       Date:  1977-03-21       Impact factor: 4.849

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  1 in total

1.  Homozygous hydroxymethylbilane synthase knock-in mice provide pathogenic insights into the severe neurological impairments present in human homozygous dominant acute intermittent porphyria.

Authors:  Makiko Yasuda; Lin Gan; Brenden Chen; Chunli Yu; Jinglan Zhang; Miguel A Gama-Sosa; Daniela D Pollak; Stefanie Berger; John D Phillips; Winfried Edelmann; Robert J Desnick
Journal:  Hum Mol Genet       Date:  2019-06-01       Impact factor: 6.150

  1 in total

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