Federico Oppi

Are subjective cognitive complaints a risk factor for dementia

The objective is to evaluate the prognosis of subjective cognitive complaints (SCC) patients during 4-year follow-up. A prospective study on 92 SCC patients investigating their cognitive, affective and behavioural aspects. SCC patients were classified as having no objective cognitive impairment (NOCI), mild cognitive impairment (MCI), or subtypes of MCI. Results: 43 patients were found to have NOCI and 49 MCI. During the follow-up, 45.5% of NOCI patients remained unchanged, 13.9% were diagnosed as MCI and only one progressed to dementia. Of the MCI patients, 32.3% remained stable, 18.4% became demented and 4% reverted to NOCI. Visual attention, behavioural memory, long-term verbal memory, apathy and caregiver distress, provided independent predictors of progression to dementia.

Accelerated long-term forgetting in temporal lobe epilepsy: Evidence of improvement after left temporal pole lobectomy

Accelerated long term forgetting (ALF) is a characteristic cognitive aspect in patients affected by temporal lobe epilepsy that is probably due to an impairment of memory consolidation and retrieval caused by epileptic activity in hippocampal and parahippocampal regions. We describe a case of a patient with TLE who showed improvement in ALF and in remote memory impairment after an anterior left temporal pole lobectomy including the uncus and amygdala. Our findings confirm that impairment of hippocampal functioning leads to pathological ALF, whereas restoration of hippocampal functioning brings ALF to a level comparable to that of controls.

Cognitive and Sleep Features of Multiple System Atrophy: Review and Prospective Study

Background: The profile and degree of cognitive impairment in Multiple System Atrophy (MSA) and the impact of sleep disorders, REM sleep behavior disorder (RBD) in particular, in parkinsonism-related cognitive deficits are currently being debated. Summary: We reviewed the cognitive, affective and sleep findings in MSA and also carried out a longitudinal investigation of 10 MSA patients. At the first evaluation, 3 patients showed isolated cognitive deficits. After a mean of 16 months, these patients remained unchanged, while 1 patient worsened from a normal condition. No significant differences emerged when the cognitive, affective and video-polysomnographic findings of MSA-P and MSA-C were compared. Depression was present in half of the patients, although it did not influence their cognitive performance. Comparisons between the first and second evaluation data showed significant worsening in visual attention and in ADL/IADL and UMSARS. Key Messages: Isolated cognitive deficits are evidenced in a minority of MSA patients with the absence of a clear-cut diagnosis of dementia in the early stages of the disease. Attention and executive functions are often impaired. This study with a short follow-up period showed that RBD, although present in almost all patients affected by MSA, does not appear a clear early marker of cognitive impairment. Future longer-term studies with a larger patient sample are thus encouraged.

Relationship of white and gray matter abnormalities to clinical and genetic features in myotonic dystrophy type 1

Background Myotonic dystrophy type 1 (DM1) represents a multisystemic disorder in which diffuse brain white and gray matter alterations related to clinical and genetic features have been described. We aimed to evaluate in the brain of adult patients with DM1 (i) white and gray matter differences, including cortical-subcortical gray matter volume and cortical thickness and (ii) their correlation with clinical disability, global neuropsychological performance and triplet expansion. Methods We included 24 adult genetically-confirmed DM1 patients (14 males; age: 38.5 ± 11.8 years) and 25 age- and sex-matched healthy controls (14 males; age: 38.5 ± 11.3 years) who underwent an identical brain MR protocol including high-resolution 3D T1-weighted, axial T2 FLAIR and DTI sequences. All patients underwent an extensive clinical and neuropsychological evaluation. Voxel-wise analyses of white matter, performed by using Tract Based Spatial Statistics, and of gray matter, with Voxel-based Morphometry and Cortical Thickness, were carried out in order to test for differences between patients with DM1 and healthy controls (p < 0.05, corrected). The correlation between MRI measures and clinical-genetic features was also assessed. Results Patients with DM1 showed widespread abnormalities of all DTI parameters in the white matter, which were associated with reduced gray matter volume in all brain lobes and thinning in parieto-temporo-occipital cortices, albeit with less extensive cortical alterations when congenital cases were removed from the analyses. White matter alterations correlated with clinical disability, global cognitive performance and triplet expansions. Conclusion In patients with DM1, the combined smaller overall gray matter volume and white matter alterations seem to be the main morpho-structural substrates of CNS involvement in this condition. The correlation of white matter differences with both clinical and genetic findings lends support to this notion.

The Tree-Drawing Test (Koch’s Baum Test): A Useful Aid to Diagnose Cognitive Impairment

Objective. To study the Tree-Drawing Test in a group of demented patients and compare it with a group of mild cognitively impaired patients (MCI) and controls. Methods. Consecutive outpatients were classified as affected by dementia (Alzheimers disease (AD), frontotemporal dementia (FTD), and vascular dementia (VD)) or by MCI. Patients and controls underwent the Tree-Drawing Test and MMSE. Results. 118 AD, 19 FTD, 46 VD, and 132 MCI patients and 90 controls were enrolled. AD patients draw trees globally smaller than other patients and controls. FTD patients draw trees with a wider space occupation than AD and MCI patients but smaller than controls as well as VD patients. Trees drawn by MCI patients are intermediate in size between AD patients and controls. The trunk-to-crown ratio of trees drawn by cognitive impaired patients is greater than controls while the tree size-relative-to-page space index is significantly smaller. The tree size-relative-to-page space index of trees drawn by AD patients is smaller than that of the other cognitively impaired patients. Tree height and the trunk-to-crown ratio are independent predictors of cognitive impairment. Conclusions. Trees drawn by cognitively impaired patients are different from those drawn by healthy subjects with a progressive differentiation from mild to more relevant degrees of cognitive impairment.

Simple verbal analogies test: normative data on a short task exploring abstract thinking

Background and aimsThe simple verbal analogies test (SVAT) is a short neuropsychological task requiring few minutes of administration that explores inductive verbal abstract thinking. It already showed a good specificity and sensitivity in discriminating normal controls from probable Alzheimer’s disease patients. Verbal working memory, semantic knowledge and memory and word-finding ability are also involved in performing analogies. The aim of this study is to provide the normative values of this test in a sample of normal controls and corrections of raw scores and equivalent scores.Methods and resultsWe determined the normative values of SVAT in a sample of 424 normal controls to provide corrections of raw scores and equivalent scores.ConclusionsSVAT is a useful test to assess executive functions, working memory and to discriminate between cognitive deterioration and normal aging.

Speech apraxia or aphemia

Josephs recently reported interesting data on frontotemporal dementia and related disorders. In defining the different terminologies associated with progressive aphasias, the author used the term apraxia of speech, which is characterized by slow and impaired articulation, dysprosody, phonetic or phonemic errors, and frequent oral apraxia. We believe that, for this condition, it is preferable to use the term aphemia. This disturbance of speech is frequently associated with an orofacial apraxia that is not present in all reported patients and that cannot explain all of the symptoms. In fact, the frequent phonemic paraphasias and paragraphias cannot be interpreted as an apraxic disorder. Furthermore, writing errors were due to articulatory suppression as was demonstrated in healthy control subjects, thereby excluding a linguistic basis. Aphemia is a rare disorder of speech without a global intellectual deficit caused by a focal degeneration of the anterior frontal regions, mainly of the frontal operculum, included into cases of primary slowly progressive focal cognitive deficits. This clinical picture can have different outcomes with possible progression to a primary motor aphasia, frontotemporal dementia, or both. The aphasic, apraxic, or dysarthric nature of aphemia is debatable; however, the selective articulatory dysfunction of prevalent cortical origin appears most convincing. Nevertheless, it is probable that aphemia represents a syndrome that has to be better defined in its clinical, pathological, and genetic aspects.

Human figure drawing distinguishes Alzheimer’s patients: a cognitive screening test study

To study human figure drawing in a group of Alzheimers disease (AD) patients and compare it with a group of patients with mild cognitive impairment (MCI) and controls. We evaluated consecutive outpatients over a one-year period. Patients were classified as affected by AD or by MCI. All patients and controls underwent a simplified version of the human-figure drawing test and MMSE. A qualitative and quantitative analysis of all human figures was obtained. 112 AD, 100 MCI patients and 104 controls were enrolled. AD patients drew human figures poor in details and globally smaller than MCI patients and controls. Human figures drawn by MCI patients are intermediate in body height between those of the AD patients and the healthy subjects. The head-to-body ratio of human figures drawn by AD patients is greater than controls and MCI patients, while the human figure size-relative-to-page space index is significantly smaller. Body height is an independent predictor of cognitive impairment correlating with its severity and with the number of the figures details. Human figures drawn by AD patients are different from those drawn by healthy subjects and MCI patients. Human figure drawing test is a useful tool for orienting cognitive impairments diagnosis.

Precuneal Thickness and Depression in Parkinson Disease

Background: Depression-related gray matter changes in Parkinson disease (PD) patients have been reported, although studies investigating cortical thickness in early-stage disease are lacking. Objective: We aimed to evaluate cortical changes related to depression in early-stage PD patients with an extensive neuropsychological evaluation. Methods: 17 PD patients and 22 healthy controls underwent a 1.5-T brain MR protocol, and voxel-wise differences in cortical thickness among patients with (n = 6) and without (n = 11) depression and controls were evaluated using FreeSurfer software. Results: Cortical thickness was increased in the precuneus bilaterally in PD patients with depression compared to the other groups (number of vertices >100; p < 0.001, uncorrected) with a direct correlation with the Beck Depression Inventory score (p < 0.001, uncorrected). Conclusion: Precuneal cortical thickening is evident in PD patients with mild-moderate depression even in the early stages of the disease. This finding may reflect the early involvement of this region in the development of PD-related depression.

Superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus: a case report

We describe the case of a man whose initial clinical presentation included sensorineural hearing loss and orthostatic hypotension. The patient was diagnosed with superficial siderosis associated with peripheral autonomic failure and tetraventricular hydrocephalus.