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81. Neuropsychiatric Features in Motor Neurone Disease (MND)vs. behavioural variant Frontotemporal Dementia (bvFTD)Patricia Lillo, Eneida Mioshi, John R. Hodges
Prince of Wales Medical Research Institute, University of NSW
Aim: To compare the profile of behavioural features in patientswith motor neurone disease (MND) vs. patients with behaviouralvariant Frontotemporal Dementia (bvFTD).
Participants and methods: 81 carers of MND patients and 25carers of bvFTD patients (NSW) completed the revised version ofthe Cambridge Behavioural Inventory (CBI-R). This scale is a 45-itemcarer based questionnaire, investigating cognitive abilities, everydayfunctional ability and neuropsychiatric symptoms in dementia.
Results: according to carer reports, 60% of MND patients hadabnormal eating habits, abnormal behaviour and stereotypical andmotor behaviour. Notably, reduced motivation was reported in 84%of the MND cases, with almost 60% of them having moderate-severeproblems. The absence of abnormal beliefs was striking, being pres-ent in only 5% of the cases. The proportion of patients presentingbehavioural problems was significant higher in the bvFTD groupthan MND in the following domains: memory & orientation, eatinghabits, abnormal behaviour, stereotypic & motor behaviour, motiva-tion, and beliefs (p < 0.001). Conversely, and probably due to themotor involvement, self care (p < 0.013) and sleep (p > 0.05) weremore affected in MND patients.
Conclusions: As expected, the proportion of patients with moder-ate-severe endorsement on the CBI-R was higher in the bvFTD group.However, the proportion of MND patients with behavioural changesreached more than 50% and presented a very distinctive pattern,being reduced motivation the most prominent feature.
doi:10.1016/j.jocn.2010.07.082
82. Amour et neurologique: Transient global amnesia followingsexual intercourseAbhishek Malhotra, Scott Whyte, Johnathan Sturm, Denis Crimmins
Department of Neurology, Gosford Hospital, NSW
Introduction
Transient Global Amnesia (TGA) is characterized by sudden onsetof anterograde and retrograde amnesia which resolves within 24hours. There is often a precipitating event including physical exer-tion or emotional stress. The pathophysiology is still unclear buthypotheses include medial temporal dysfunction resulting fromraised venous pressure causing venous congestion of hippocampus.The diagnosis of TGA is made on clinical grounds. Recently describedMRI findings of focal diffusion lesions in the CA1 field of hippocam-pal cornu ammonis can support the diagnosis.
Case reports
We report five cases of TGA precipitated by sexual intercourse inpatients aged between 55 and 64 years. Four of the patients werefemale and two of them experienced recurrent episodes. All patientshad EEG and cerebral imaging to rule out an alternate diagnosis.
Discussion
TGA is often associated with an antecedent precipitant. Sexualintercourse has been increasingly reported in the literature as a
provocative factor. In the event of recurrent episodes, counsellingthe patient regarding the intercourse technique may be helpful,with emphasis on reducing the amount of effort put into theevent. Investigations are occasionally needed to exclude the differ-ential diagnosis that includes posterior circulation ischaemia,complex partial seizures and transient epileptic amnesia amongothers.
References
1. Lewis SL. Aetiology of transient global amnesia. Lancet1998;352:397–9.
2. Miller Fisher C. Transient global amnesia: precipitating activitiesand other observations. Arch Neurol 1982;39:605–8.
3. Gallagher J, Murphy MS, Carroll J. Transient global amnesia aftersexual intercourse. Ir J Med Sci 2005;174:86–7.
4. Bartsch T, Deuschl G. Transient global amnesia: functionalanatomy and clinical implications. Lancet Neurol 2010;9:205–14.
doi:10.1016/j.jocn.2010.07.083
83. A case of cerebral vasculitis due to giant cell arteritisAndrew W. Moey a, Thomas Kimber a, Peter Blumbergs b
a Royal Adelaide Hospital, SAb Institute of Medical and Veterinary Science, SA
Objective: To report a rare case of biopsy-proven giant cell arter-itis (GCA) involving the intracerebral vessels, and to highlight theneed for early and aggressive immunosuppressive treatment.
Method/results: The case is a 78 year old woman whopresented with a subacute history of headache and monocularblindness due to anterior ischaemic optic neuropathy. CT and con-ventional angiography showed changes consistent with cerebralvasculitis and superficial temporal artery biopsy confirmed GCA.Despite treatment with high dose corticosteroids, she developeda progressive left hemiplegia and MRI showed multiple regionsof acute ischaemia in both cerebral hemispheres. Pulse intravenouscyclophosphamide was then added, after which her conditionstabilised.
Conclusions: This case highlights several issues:
1. In rare cases, GCA can cause an aggressive cerebral vasculitis.2. Non-invasive imaging of the cerebral circulation (eg. CTA, MRA)
should be considered in cases of biopsy-proven GCA.3. If imaging findings consistent with cerebral vasculitis are found,
early and aggressive immunosuppressant treatment should begiven.
4. Even with such treatment, the outcome may be poor.
doi:10.1016/j.jocn.2010.07.084
84. Dural ectasia in Marfan’s syndrome: Clinical and MRI featuresKatrina A. Morris, John Ly, Susan Tomlinson
St Vincent’s Hospital, NSW
Background: Marfan’s Syndrome is an autosomal dominant disor-der of elastic connective tissue resulting from mutations in the FBN1
1634 Abstracts / Journal of Clinical Neuroscience 17 (2010) 1610–1638