PubMed · 12449123
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Denise O'Brien. 2002. Delirium update.. https://doi.org/10.1097/00000446-200207000-00007
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Patients with neurologic disease who require surgery present distinct issues and challenges for the medical consultant. Although it is not possible to offer a unified approach to neurologic patients, the primary care consultant should understand the clinical issues that are common to these patients, and the individual considerations necessitated by the nature of the neurologic disorder and the clinical characteristics of the patient. The preoperative evaluation combines elements of literature evidence on risk assessment with a thorough understanding of the planned procedure and local practice patterns, and clinical judgment as to the estimated risk-benefit ratio. Perioperative management necessitates attention to many general principles of perioperative care, such as awareness of the potential for cardiopulmonary complications and the need for DVT prophylaxis. In addition, there are management issues for neurologic patients, such as blood pressure control and evaluation of hyponatremia, which may differ from other surgical patients. In these circumstances, the interaction of the neurologic condition with the medical condition and the implications of treatment on the underlying neurologic process also need to be considered.
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BACKGROUND: Machado-Joseph disease (MJD; spinocerebellar ataxia type 3) is a hereditary neurodegenerative disease caused by mutation of the MJD1 gene. Patients with MJD usually present with cerebellar ataxia, external ophthalmoplegia, pyramidal and extrapyramidal signs, and muscle wasting. However, it has been reported that these patients do not demonstrate dementia. CASE DESCRIPTION: We noticed symptoms of dementia and delirium in 4 patients with MJD. The symptoms included abnormal behavior, excitation, an uncooperative attitude, crying, disorientation, slow thought processes, hallucinations, and delusions. These symptoms were observed in patients with a relatively young onset age, and after a long clinical course. In these patients, the CAG repeat length in the MJD1 gene was much longer compared with the mean repeat length found in patients with MJD. On electroencephalographical examination, they showed slow background activity, but computed tomography and magnetic resonance imaging scans showed no cerebrocortical atrophy. Neuropathological findings in 2 patients revealed a normal cortical structure on conventional morphological examination, but at immunohistochemical examination, we found abnormal staining by an antipolyglutamine antibody in the cerebrocortical neuronal nuclei. CONCLUSIONS: Symptoms of dementia and delirium in patients with MJD could occur in the late stages, and they might be caused not by loss of cerebrocortical neurons, but by their dysfunction.