Tertiary stage

Hypotonia and hyporeflexia develop and the terminal state is marked by flaccid muscle weakness. Plantar responses remain flexor and abdominal reflexes intact. Progressive dysphagia occurs, and patients become incontinent of urine and faeces. Inanition and emaciation develop. Transient conjugate eye signs and dementia may occur. Primitive reflexes develop in occasional cases. Brainstem involvement and both bulbar and pseudobulbar signs occur. Respiratory failure and bronchopneumonia eventually lead to death. The tertiary stage lasts 1 to 2 months.

Iatrogenic Creutzfeldt-Jakob disease

Iatrogenic transmission of CJD has occurred by accidental inoculation with human prions as a result of medical procedures. Such iatrogenic routes include the use of inadequately sterilized neurosurgical instruments, dura mater and corneal grafting, and use of human cadaveric pituitary-derived growth hormone or gonadotrophin. It is of considerable interest that cases arising from intracerebral or optic inoculation manifest clinically as classical CJD, with a rapidly progressive dementia, while those resulting from peripheral inoculation, most notably following pituitary-derived growth hormone exposure, typically present with a progressive cerebellar syndrome, and are in that respect somewhat reminiscent of kuru. Unsurprisingly the incubation period in intracerebral cases is short (19-46 months for dura mater grafts) as compared with peripheral cases (typically 15 years or more). There is evidence for genetic susceptibility to iatrogenic CJD with an excess of codon 129 homozygotes(13) (see discussion of aetiology above).

Epidemiological studies have not shown increased risks of particular occupations that may be exposed to human or animal prions, although individual CJD cases in two histopathology technicians, a neuropathologist, and a neurosurgeon have been documented. While there have been concerns that CJD may be transmissible by blood transfusion, extensive epidemiological analysis in the United Kingdom has found that the frequency of blood transfusion and donation was no different in over 200 cases of CJD and a matched control population. tyf) Recipients of blood transfusions who developed CJD had clinical presentations similar to those of sporadic CJD patients and not to the more kuru-like iatrogenic cases arising from peripheral exposure to human prions. Furthermore, experimental transmission studies have shown only weak evidence for infectivity in blood,(45) even when inoculated via the most efficient (intracerebral) route. It cannot be assumed that the same picture will hold for vCJD as this is caused by a distinct prion strain (18> from those causing classical CJD and has a distinct pathogenesis. (46> It is also conceivable that many individuals could currently be incubating this disease.

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