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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Adult polyglucosan body disease associated with an extrapyramidal
syndrome</title>
<author><name sortKey="Robertson, N" sort="Robertson, N" uniqKey="Robertson N" first="N" last="Robertson">N. Robertson</name>
</author>
<author><name sortKey="Wharton, S" sort="Wharton, S" uniqKey="Wharton S" first="S" last="Wharton">S. Wharton</name>
</author>
<author><name sortKey="Anderson, J" sort="Anderson, J" uniqKey="Anderson J" first="J" last="Anderson">J. Anderson</name>
</author>
<author><name sortKey="Scolding, N" sort="Scolding, N" uniqKey="Scolding N" first="N" last="Scolding">N. Scolding</name>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">9810960</idno>
<idno type="pmc">2170328</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2170328</idno>
<idno type="RBID">PMC:2170328</idno>
<date when="1998">1998</date>
<idno type="wicri:Area/Pmc/Corpus">000191</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000191</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Adult polyglucosan body disease associated with an extrapyramidal
syndrome</title>
<author><name sortKey="Robertson, N" sort="Robertson, N" uniqKey="Robertson N" first="N" last="Robertson">N. Robertson</name>
</author>
<author><name sortKey="Wharton, S" sort="Wharton, S" uniqKey="Wharton S" first="S" last="Wharton">S. Wharton</name>
</author>
<author><name sortKey="Anderson, J" sort="Anderson, J" uniqKey="Anderson J" first="J" last="Anderson">J. Anderson</name>
</author>
<author><name sortKey="Scolding, N" sort="Scolding, N" uniqKey="Scolding N" first="N" last="Scolding">N. Scolding</name>
</author>
</analytic>
<series><title level="j">Journal of Neurology, Neurosurgery, and Psychiatry</title>
<idno type="ISSN">0022-3050</idno>
<idno type="eISSN">1468-330X</idno>
<imprint><date when="1998">1998</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><p>A 50 year old patient is described who presented with
parkinsonism, frontal dementia, peripheral neuropathy, neurogenic
bladder, and upper motor neuron signs. No improvement in objective
measurements of extrapyramidal dysfunction were seen with an
incremental apomorphine test or more prolonged oral dopamine challenge.
Neurophysiology disclosed changes compatible with a diffuse axonal
neuropathy and pathological examination of a length of sural nerve
taken at biopsy showed multiple polyglucosan bodies characteristic of adult polyglucosan body disease (APGBD). This case underlines the
diverse clinical presentation of this rare neurological disease and the
importance of recognising the unusual association of clinical features
in making the diagnosis. APGBD should be included in the differential
diagnosis of parkinsonism unresponsive to dopaminergic therapy.
</p>
</div>
</front>
</TEI>
<pmc article-type="brief-report"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">J Neurol Neurosurg Psychiatry</journal-id>
<journal-title>Journal of Neurology, Neurosurgery, and Psychiatry</journal-title>
<issn pub-type="ppub">0022-3050</issn>
<issn pub-type="epub">1468-330X</issn>
<publisher><publisher-name>BMJ Group</publisher-name>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">9810960</article-id>
<article-id pub-id-type="pmc">2170328</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Short Report</subject>
</subj-group>
</article-categories>
<title-group><article-title>Adult polyglucosan body disease associated with an extrapyramidal
syndrome</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Robertson</surname>
<given-names>N</given-names>
</name>
</contrib>
<contrib contrib-type="author"><name><surname>Wharton</surname>
<given-names>S</given-names>
</name>
</contrib>
<contrib contrib-type="author"><name><surname>Anderson</surname>
<given-names>J</given-names>
</name>
</contrib>
<contrib contrib-type="author"><name><surname>Scolding</surname>
<given-names>N</given-names>
</name>
</contrib>
</contrib-group>
<aff>University of Cambridge Neurology Unit, Addenbrooke's Hospital, UK.</aff>
<pub-date pub-type="ppub"><month>11</month>
<year>1998</year>
</pub-date>
<volume>65</volume>
<issue>5</issue>
<fpage>788</fpage>
<lpage>790</lpage>
<self-uri xlink:role="pdf" xlink:type="simple" xlink:href="http://jnnp.bmj.com/cgi/reprint/65/5/788.pdf"></self-uri>
<self-uri xlink:role="abstract" xlink:type="simple" xlink:href="http://jnnp.bmj.com/cgi/content/abstract/65/5/788"></self-uri>
<self-uri xlink:role="fulltext" xlink:type="simple" xlink:href="http://jnnp.bmj.com/cgi/content/full/65/5/788"></self-uri>
<abstract><p>A 50 year old patient is described who presented with
parkinsonism, frontal dementia, peripheral neuropathy, neurogenic
bladder, and upper motor neuron signs. No improvement in objective
measurements of extrapyramidal dysfunction were seen with an
incremental apomorphine test or more prolonged oral dopamine challenge.
Neurophysiology disclosed changes compatible with a diffuse axonal
neuropathy and pathological examination of a length of sural nerve
taken at biopsy showed multiple polyglucosan bodies characteristic of adult polyglucosan body disease (APGBD). This case underlines the
diverse clinical presentation of this rare neurological disease and the
importance of recognising the unusual association of clinical features
in making the diagnosis. APGBD should be included in the differential
diagnosis of parkinsonism unresponsive to dopaminergic therapy.
</p>
</abstract>
</article-meta>
</front>
</pmc>
</record>
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