Marta Fernández-Matarrubia

Epicrania Fugax: 19 Cases of an Emerging Headache

Epicrania fugax (EF) is a primary headache of recent description. We aimed to report 19 new cases of EF, and thus contribute to the characterization of this emerging headache.

Prevalence of migraine in patients with restless legs syndrome: a case-control study.

To assess and compare the prevalence of migraine in patients with restless legs syndrome (RLS) and matched controls.

The Hayling Test: Development and Normalization of the Spanish Version

OBJECTIVE The Hayling Sentence Completion Test evaluates the ability to inhibit an automatic response. It has also been suggested for the assessment of orbitofrontal cortex function. The aim of the study was to develop a Spanish version of the Hayling test and to obtain normative data. METHOD Responses to 60 sentences from 50 healthy controls were used to develop the task. Additionally, 185 healthy controls aged between 18 and 99 years were examined with the test in order to obtain normative data. The overlapping interval strategy was used to maximize the sample size. Age- and education-adjusted scores were obtained using linear regression analysis. RESULTS Age and educational level had a significant effect on the different scores. Good internal reliability and inter-rater variability were observed. CONCLUSIONS We provide normative data adjusted for age and education. Our results enable the use of this test for clinical and research purposes in the field of neuropsychological assessment.

Different apathy clinical profile and neural correlates in behavioral variant frontotemporal dementia and Alzheimer's disease

Apathy is one of the most common and disabling syndromes of dementia. Clinical apathy expression and neuroanatomical basis of apathy seem to differ between behavioral variant frontotemporal dementia (bvFTD) and Alzheimers disease (AD), although evidence is scarce and poorly understood. Our main purposes were to compare the clinical apathy profile from patients with bvFTD and AD and analyze the relationship between apathy and brain metabolism measured using positron emission tomography imaging with 18F fluorodeoxyglucose (FDG‐PET).

Validation of the Spanish Version of the LASSI-L for Diagnosing Mild Cognitive Impairment and Alzheimer’s Disease

BACKGROUND The Loewenstein-Acevedo Scale for Semantic Interference and Learning (LASSI-L) is a novel cognitive test that measures recovery from proactive semantic interference, which may be an early cognitive marker of Alzheimers disease (AD). OBJECTIVE To generate normative data for a Spaniard population and to validate the LASSI-L for the diagnosis of amnestic mild cognitive impairment (aMCI) and mild AD. METHODS We performed a cross-sectional study in which 97 healthy participants, 34 with aMCI, and 33 with mild AD were studied with LASSI-L and a comprehensive neuropsychological protocol. The overlapping strategy analysis was used to maximize the sample size and to provide age- and education-adjusted normative data using a logistic regression analysis. RESULTS Internal consistency was 0.932. Convergent validity with the Free and Cued Selective Reminding Test was moderate. LASSI-L raw scores were correlated with age and years of education, but not gender. The area under the curve for discriminating between healthy controls and aMCI was 0.909, and between healthy controls and mild AD was 0.986. LASSI-L sub-scores representing maximum storage capacity, recovery from proactive interference, and delayed recall yielded the highest diagnostic accuracy. CONCLUSIONS The LASSI-L is a reliable and valid test for the diagnosis of aMCI and mild AD. The age and education influences on the performance of the test and normative data are provided. LASSI-L merits further studies to evaluate its ability to detect preclinical AD and predict progression to aMCI and early dementia.

Addenbrooke's cognitive examination III: diagnostic utility for mild cognitive impairment and dementia and correlation with standardized neuropsychological tests.

BACKGROUND Addenbrookes Cognitive Examination III (ACE-III) is a screening test that was recently validated for diagnosing dementia. Since it assesses attention, language, memory, fluency, and visuospatial function separately, it may also be useful for general neuropsychological assessments. The aim of this study was to analyze the tools ability to detect early stages of Alzheimers disease and to examine the correlation between ACE-III scores and scores on standardized neuropsychological tests. METHODS Our study included 200 participants categorized as follows: 25 healthy controls, 48 individuals with subjective memory complaints, 47 patients with amnestic mild cognitive impairment and 47 mild Alzheimers disease, and 33 patients with other neurodegenerative diseases. RESULTS The ACE-III memory and language domains were highly correlated with the neuropsychological tests specific to those domains (Pearson correlation coefficient of 0.806 for total delayed recall on the Free and Cued Selective Reminding Test vs. 0.744 on the Boston Naming Test). ACE-III scores discriminated between controls and patients with amnestic mild cognitive impairment (AUC: 0.906), and between controls and patients with mild Alzheimers disease (AUC: 0.978). CONCLUSION Our results suggest that ACE-III is a useful neuropsychological test for assessing the cognitive domains of attention, language, memory, and visuospatial function. It also enables detection of Alzheimers disease in early stages.

Validation of the Lille's Apathy Rating Scale in Very Mild to Moderate Dementia.

OBJECTIVE Apathy is one of the most common and disabling syndromes of dementia and presents at all stages of the disease. Comprehensive and structured methods to assess apathy in dementia are still needed. Lilles Apathy Rating Scale (LARS) has shown good psychometric properties for apathy evaluation in Parkinson disease but has not been validated in dementia. The aim of this study was to validate the LARS in a cohort of patients with very mild to moderate dementia. METHODS 101 patients with cognitive impairment (Clinical Dementia Rating ≤ 2) and 50 healthy subjects were recruited. Patient diagnoses included 43 individuals with Alzheimer disease, 41 frontotemporal dementia, and 17 primary progressive aphasia. In addition to LARS, the following assessments were administered: Clinical Dementia Rating, Interview for Deterioration in Daily Living Activities in Dementia, Functional Activities Questionnaire, Frontal Behavioral Inventory, Neuropsychiatric Inventory (NPI), and Hamilton Depression Rating Scale. RESULTS Internal consistency for LARS (Cronbachs alpha) was 0.940. Test-retest intraclass correlation coefficient (ICC) was 0.940 and inter-rater ICC was 0.987. The correlation among LARS and NPI apathy scores (concurrent validity) was 0.834. Receiver operating characteristic analysis estimated an area under the curve of 0.987. The optimal cutoff point was -10. Although total LARS score was influenced by the presence of depression, this disorder was independent with respect to apathy. CONCLUSION LARS is reliable and valid for detecting and quantifying apathy in patients with dementia, even in very early stages of the disease.

Episodic Memory Dysfunction in Behavioral Variant Frontotemporal Dementia: A Clinical And FDG-PET Study

BACKGROUND Episodic memory disturbance is still considered as an exclusion criterion for behavioral variant frontotemporal dementia (bvFTD), but growing evidence suggests that memory can be impaired. OBJECTIVE Our main purposes were to assess episodic memory in a group of bvFTD patients comparatively with Alzheimers disease (AD) patients, and analyze the relationship between episodic memory and brain metabolism measured using positron emission tomography imaging with 18F-fluorodeoxyglucose (FDG-PET). METHODS Twenty-six bvFTD, 29 AD, and 24 healthy controls were included. Episodic memory was assessed by the Free and Cued Selective Reminding Test (FCSRT), which controls for effective encoding and measures memory consolidation processing. All participants underwent FDG-PET brain scans to provide data for voxel-based brain mapping analysis. RESULTS Half of bvFTD patients had a deficit of total, free delayed, and total free delayed recall as severe as AD patients (amnestic-FTD). The other half had FCSRT scores similar to controls (non-amnestic-FTD). Imaging analyses revealed that amnestic-FTD showed bilateral lower metabolism than non-amnestic-FTD in anterior parahippocampal and inferior temporal gyri. Additionally, FCSRT total and total delayed scores were inversely correlated with parahippocampal metabolism in both bvFTD and AD. Besides, bvFTD showed an inverse association among FCSRT and inferior temporal metabolism. CONCLUSIONS Our findings support that bvFTD could present a genuine amnesia affecting storage and consolidation abilities, which involves structures implicated in the Papez circuit, as occurs in AD, and also inferior temporal regions. These results contribute to understanding the mechanisms underpinning memory dysfunction in bvFTD, and may be relevant to further revisions of the current diagnostic criteria.

Case report: Epicranial pain after radiotherapy for skull base meningioma – the first symptomatic epicrania fugax?

Background To date, no symptomatic forms of epicrania fugax (EF) have been reported. Here, we describe the first EF-type pain to be probably caused by an underlying disorder. Case report A 77-year-old woman started suffering from left V1–V2 trigeminal neuralgia at 72 years of age. Neurologic examination was normal. Magnetic resonance imaging (MRI) showed a left middle sphenoid wing meningioma compressing the left trigeminal nerve medially. After trying several neuromodulators, she received stereotactic radiotherapy. One month later, the episodes of facial pain were significantly diminished, but she started feeling brief electric paroxysms across her left hemicranium that were clinically identical to those of backwards EF. Serial MRI showed persistence of the meningioma without changes. Conclusion Although the pathogenesis of EF remains uncertain, this case is consistent with a symptomatic origin in the trigeminal root/pathway. The onset of the EF-like pain could have been caused by the compressive effect of the tumour or, most likely, by the radiation.

Primary progressive aphasia with occipital impairment.

Primary progressive aphasia (PPA) is a clinical entity characterized by a neurodegenerative language disorder. The current classification in three variants of PPA has improved the ability to predict the pathological substrate (1,2).Wicklund et al. have recently described two clinical cases of progressive aphasia with left occipitotemporal hypometabolism, suggesting an overlapping formwith posterior cortical atrophy (PCA), a subtype of focal onset of Alzheimers disease (3). We present two cases of progressive aphasiawith left parieto-temporo-occipital hypometabolism in fluorodeoxyglucose positron emission tomography (F-FDG-PET), and amyloid deposition in PET imaging with F-Florbetapir. These two cases support the existence of a subtype of primary progressive aphasia with associated left occipital lobe impairment. In this article, we discuss the clinical significance of this variant. The first case was a right-handed 69-year-old woman with below basic literacy level. She developed a word finding difficulty 3 years ago, with no other associated symptomatology. Her speech had a qualitative reduction in fluency, with anomia and phonemic paraphasias, and a disturbance in the repetition of long phrases and nonwords. Her cognitive symptoms progressed slowly, and she developed global dementia over 2 years later. During this period she never presented Parkinsonism, hallucinations or REM behavior disorder. Moreover, she showed a normal uptake of I-ioflupane using single-photon emission computed tomography. A F-FDG-PET performed 3 years after clinical onset showed left occipito-temporo-parietal hypometabolism. 5 years after clinical onset, a second F-FDG-PET showed a more pronounced left occipitotemporo-parietal hypometabolism, and PET imagingwith F-Florbetapir revealed diffused amyloid deposition (Fig. 1). The second case was a right-handed 70-year-old man, nonprofessional painter. He presented with a 6-month history of wordfinding difficulty, without any other symptom and keeping his painting skills unimpaired. On the neuropsychological examination he reached 29 over 30 in the Minimental State Examination and 73 over 100 in the Addenbrookes Cognitive Examination (4). The language examination showed anomia in confrontation naming (Boston Naming Test 26 over 60; 34 phonemic cueswere provided, and the patient correctly answered in 22), without agramatism and unimpaired repetition and semantic knowledge. Visuospatial and visuoperceptive skillswere impaired (judgment of line orientation 13 over 30; visual object and space perception