Exploded drawing in posterior cortical atrophy.
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Biomedical subjects
Paul McMonagle
Publications and source records attributed to Paul McMonagle.
Corticobasal degeneration and progressive aphasia.
OBJECTIVE: To describe language impairment in the corticobasal degeneration syndrome (CBDS) presenting as either a cognitive or motor disorder, to compare the evolution of aphasia in CBDS with primary progressive aphasia (PPA), and to examine whether the side of maximal cerebral atrophy or akinesia reflects the severity of aphasia. METHODS: We divided 40 patients with CBDS according to motor or cognitive onsets and conducted detailed language assessments with the Western Aphasia Battery (WAB). We analyzed scores according to the side of atrophy and motor rigidity. Longitudinal performance over three annual assessments was compared against matched patients with PPA and Alzheimer disease. RESULTS: Language at baseline was more impaired in cognitive than motor-onset CBDS but there was no correlation between the side of atrophy or motor impairment and the WAB. Serial assessment (n = 19) showed a similar evolution of aphasia in cognitive-onset CBDS and PPA and delayed aphasia in motor-onset CBDS. CONCLUSION: Aphasia is common in the corticobasal degeneration syndrome but there is little correlation with the laterality of clinical deficits. Cognitive-onset corticobasal degeneration syndrome and primary progressive aphasia are similar such that their aphasia appears identical.
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The cognitive profile of posterior cortical atrophy.
BACKGROUND: Posterior cortical atrophy (PCA) is a progressive dementia characterized by prominent disorders of higher visual processing, affecting both dorsal and ventral streams to cause Balint's syndrome, alexia, and visual agnosia. OBJECTIVE: To define the cognitive profile of PCA and compare to the typical, primary amnestic dementia of the Alzheimer's type (DAT). METHODS: The authors used standard cognitive tests and a novel battery designed to reflect dysfunction in both ventral (Object, Face & Color Agnosia Screen [OFCAS]) and dorsal (complex pictures and compound stimuli) visual streams. The authors identified 19 patients with PCA and compared their performance to a matched group of patients with DAT and normal controls. RESULTS: Patients with PCA were younger with marked impairment in visuospatial tasks, reading, and writing but relative preservation of memory compared to DAT using standard tests. Dorsal stream signs were most prevalent among the patients with PCA with no pure ventral stream syndromes found. All novel tests distinguished reliably between subjects with complex picture descriptions and processing of compound stimuli showing the most significant differences compared to DAT. CONCLUSIONS: PCA is predominantly a dorsal stream syndrome, distinct from typical DAT, which involves occipitotemporal regions over time.
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Quantitative and qualitative analyses of clock drawing in frontotemporal dementia and Alzheimer's disease.
The clock drawing test (CDT) is a widely used cognitive screening test. It is useful in identifying focal lesions and cognitive deficits in dementia groups. Lately, several studies attempted its use to differentiate between dementia subtypes. Although many studies have examined the CDT in dementia populations, research into the use of clock drawing in frontotemporal dementia (FTD) is limited. We examined quantitative (global) and qualitative (specific error type) differences on the CDT between FTD (n = 36) and Alzheimer's disease (AD; n = 25) patients and controls without dementia (n = 25). Results showed significantly lower overall scores in the dementia groups compared to the control group, whereas FTD patients scored significantly higher than the AD group. On qualitative analysis, the FTD group had fewer stimulus bound responses, conceptual deficits, and spatial or planning errors compared to the AD group. In conclusion, both global and error analysis of the CDT helped discriminate the FTD group from controls and AD patients.
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The evolution and pathology of frontotemporal dementia.
This is a clinicopathologic study of a prospective, clinic-based cohort of patients with frontotemporal dementia (FTD)/Pick complex, who were followed to autopsy. A total of 60 patients with the clinical syndromes of the behavioural variant of FTD (FTD-bv) (n = 32), primary progressive aphasia (PPA) (n = 22), corticobasal degeneration syndrome (CBDS) (n = 4) and progressive supranuclear palsy (PSP) (n = 2) at onset, referred to a cognitive neurology clinic who had subsequent post-mortem examination were included. The most common histological variety was motor neurone disease type inclusion (MNDI) (n = 18), followed by corticobasal degeneration (CBD) (n = 12), then Pick's disease (n = 6), dementia lacking distinctive histology (DLDH) (n = 6) and PSP (n = 3). Others fulfilled the histological criteria for Alzheimer's disease combined with glial pathology (n = 6), Alzheimer's disease only (n = 4), Lewy body variant (n = 2), prion disease (n = 1), vascular dementia (n = 1) and undetermined (n = 1). The most common first syndrome among the MNDI and DLDH (tau negative) pathologies was FTD-bv, but subsequently progressive aphasia (PA), occasionally CBDS and semantic dementia also developed. Tau positive histologies of CBD, PSP and Pick bodies were most frequently associated with PPA onset or CBDS/PSP, but behavioural symptoms were also common. Age of onset was earlier in tau negative cases, but the duration of illness and gender distribution were about the same in all histological variants. Although the tau negative and positive histologies are predicted to some extent by the clinical onset, the extent of the overlap and the convergence of the syndromes in the course of the disease argue in favour of maintaining the clinical and pathological varieties under a single umbrella.
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Further evidence of dementia in SPG4-linked autosomal dominant hereditary spastic paraplegia.
OBJECTIVE: To investigate the progression of cognitive impairment and its behavioral aspects in patients with SPG4-linked autosomal dominant hereditary spastic paraplegia (SPG4-ADHSP). METHODS: Sixteen patients, 45 years or older, from five families with SPG4-ADHSP were prospectively assessed. Eleven of these, from three families, were followed and 10 had two cognitive examinations using the Cambridge Cognitive Assessment (CAMCOG) 2.9 years apart. The Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE), the Neuropsychiatric Inventory (NPI), and the Nurses' Observation Scale for Geriatric Patients (NOSGER) were completed by close relatives of the 11 patients at the second assessment. Eleven matched control subjects had CAMCOG examinations 3.1 years apart. RESULTS: The mean CAMCOG score at the initial assessment was lower for the 10 HSP patients (73.5/107) than for 10 control subjects (91.7/107, p = 0.005). After 2.9 years, the HSP patients experienced a fall in the mean CAMCOG by 9.1 points to 64.4/107 (p = 0.008). The mean CAMCOG score for the control subjects (90.8/107) fell by only 0.9 points after 3.1 years (p = 0.36). The CAMCOG scores of two HSP patients moved from the mild (60 to 80) into the moderate dementia category (35 to 59) and in three other patients into the mild dementia range from borderline normal scores (81 to 85). IQCODE scores were abnormal in 9/11 HSP patients and 1/11 controls (p = 0.001). Seven of the 11 HSP patients were considered as having dementia. CONCLUSION: This study indicates an active progression of cognitive deterioration and dementia in older patients with SPG4-ADHSP.
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