Behavioral Abnormalities and Cognitive Impairment in Rare Dementia Syndromes, Progressive Supranuclear Palsy, Huntington Disease and Sporadic Creutzfeldt-Jakob Disease

Author(s):  
Ophir Keret
2010 ◽  
Vol 1 (1) ◽  
pp. 3 ◽  
Author(s):  
Vicky Chau ◽  
Sadhana Prasad ◽  
Dwight Stewart ◽  
George Heckman

Patients with Creutzfeldt–Jakob disease (CJD) may exhibit characteristic abnormalities on the electroencephalogram (EEG). However, these abnormalities have been associated with a number of cases of drug toxicity. We report a case of CJD-like syndrome associated with gabapentin. A 78-year-old man was hospitalized for recurrent falls. Three months prior to admission, gabapentin was prescribed to treat symptoms of trigeminal neuralgia. The patient subsequently presented with a two-month history of worsening gait abnormalities, negative myoclonus, and cognitive impairment. The EEG showed diffuse background slowing with larger amplitude delta discharges, which at times appeared triphasic, raising the possibility of CJD. The gait abnormalities and myoclonus resolved and the EEG normalized after the gabapentin was discontinued. Several cases of drug-induced CJD-like syndrome have been reported, mainly presenting with cognitive impairment, myoclonus, Parkinsonism, and EEG abnormalities. This patient may have been predisposed to adverse neurological effects from gabapentin owing to age, concurrent renal insufficiency, and cardiac disease. We concluded that it is imperative to include drug toxicity in the differential diagnosis of patients presenting with clinical manifestations and EEG findings suggestive of CJD, particularly in the setting of advanced age and comorbidities.


Author(s):  
J Keith-Rokosh ◽  
L C Ang

Objectives:The neuropathological findings of 32 progressive supranuclear palsy (PSP) cases over a period of 17 years were reviewed.Results:Of the 26 cases with adequate clinical data, 20 patients either presented with cognitive dysfunction or developed a cognitive impairment subsequently during the course of the disease. Co-existing changes of argyrophilic grains and corticobasal degeneration (CBD) were found in 28% and 32% of the cases respectively. Alzheimer-related pathology was found in 69% of cases but only 18.75% of cases fulfilled the consortium to establish a registry for Alzheimer's disease (CERAD) criteria for either definite or probable Alzheimer's disease. Lewy bodies were noted in four cases (12.5%), all in the subcortical regions. Only seven cases of PSP showed no pathological evidence of other co-existing neurodegenerative diseases. The severity of the cerebrovascular pathology in this cohort was insufficient to explain any clinical symptomatology.Conclusions:As in previous studies, this study has demonstrated the frequent co-existence of pathological changes usually noted in other neurodegenerative diseases in PSP. Whether these coexisting pathological changes contribute to the cognitive impairment in PSP remains uncertain.


Author(s):  
John R. Hodges

This chapter comprises 16 case histories that illustrate methods of assessment described in the rest of this book and the use of the Addenbrooke’s Cognitive Examination (ACE)-III. Each case begins with a brief history from the patient and observations by the family followed by findings on cognitive examination focusing on the profile shown on the ACE-III, the results of imaging investigations, and a discussion of the diagnosis and its differential, with a final summary of the principal conclusions, indicating whether the services of a neuropsychologist are required or not. The cases present important common conditions (such as mild cognitive impairment, Alzheimer’s disease in the mild and moderate stages, behavioural variant frontotemporal dementia, progressive non-fluent aphasia, semantic dementia, corticobasal degeneration, progressive supranuclear palsy, and Huntington’s disease) as well as interesting neuropsychological syndromes (such as prosopagnosia, amnestic stoke, and transient epileptic amnesia).


2011 ◽  
Vol 11 (2) ◽  
pp. 229-232 ◽  
Author(s):  
Katsuhiko Ogawa ◽  
Hirokazu Yoshihashi ◽  
Yutaka Suzuki ◽  
Minoru Oishi ◽  
Satoshi Kamei

Brain ◽  
2010 ◽  
Vol 133 (8) ◽  
pp. 2382-2393 ◽  
Author(s):  
R. G. Brown ◽  
L. Lacomblez ◽  
B. G. Landwehrmeyer ◽  
T. Bak ◽  
I. Uttner ◽  
...  

Author(s):  
Richard A. Walsh

Progressive supranuclear palsy is a four-repeat tauopathy that is confirmed, like all neurodegenerative disease, at postmortem examination. An expanding group of clinical syndromes are now linked with this pathology in its early stages, although with disease progression there tends to be greater clinical similarity with the classical Richardson’s syndrome, an akinetic rigid form of parkinsonism with a progressive supranuclear gaze palsy and prominent frontal cognitive impairment. Currently, there are no disease-modifying therapies for progressive supranuclear palsy; however, there continues to be interest in immunotherapies targeted at tau pathology. Liaison with colleagues with an interest in palliative neurology is appropriate for patients in the advanced stages of the disease.


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