Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease
نویسندگان
چکیده
منابع مشابه
Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease.
OBJECTIVE To investigate abnormal prion protein (PrP) deposition in the peripheral nervous system (PNS) in human prion diseases. METHODS Eight patients with prion diseases were examined: three with sporadic Creutzfeldt-Jakob disease (sCJD), two with dural graft associated CJD (dCJD), one with Gerstmann-Straussler-Scheinker disease (GSS) with a PrP P102L mutation (GSS102), and two with a P105L...
متن کاملCreutzfeldt-Jakob disease secondary to dural graft performed in 1988.
The patient was a 51-year-old male with a history of Arnold-Chiari I malformation and syringomyelia from C1 to T3. In October 1988, he was treated with laminectomy, amygdalectomy, and duraplasty. Gait alteration secondary to mild spastic paraparesis manifested as a long-term sequela. In August 2012, the patient presented symptoms of altered behavior, including irritability, mood swings and apat...
متن کاملCreutzfeldt-Jakob disease associated with peripheral neuropathy.
We describe the case of a patient with sporadic Creutzfeldt-Jakob disease (sCJD) with associated peripheral neuropathy and discuss the literature on this topic. A patient with autopsy proven CJD presented with the clinical signs of a sensory (large fibres) polyneuropathy. The neurophysiologic study showed a sensory-motor polyneuropathy with predominant axonal pattern, without denervation signs....
متن کاملPathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease.
BACKGROUND Involvement of the peripheral nervous system in the pathogenesis of prion diseases is becoming increasingly evident. However, pathologic protease-resistant prion protein deposition in the peripheral nerves of patients with Creutzfeldt-Jakob disease has never been demonstrated, to our knowledge. OBJECTIVE To determine whether mutated prion protein accumulation could be shown in the ...
متن کاملAccumulation of prion protein in the peripheral nervous system in human prion diseases.
After the finding that anti-prion antibodies stain sensory and sympathetic ganglia in variant Creutzfeldt-Jakob disease (vCJD), it was suggested that this localization supported the oral route of entry. However, prion accumulation subsequently also appeared in the peripheral nervous system (PNS) in sporadic cases. This study aims at evaluating the extent of prion protein accumulation in the PNS...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 2005
ISSN: 0022-3050
DOI: 10.1136/jnnp.2003.035154