Cognitive impairment and magnetic resonance imaging correlations in Wilson's disease.

OBJECTIVES: To evaluate the cognitive performance of a group of patients with Wilson's disease (WD) and to correlate the cognitive findings with changes in magnetic resonance imaging (MRI). METHODS: All patients with WD consecutively attended in a Movement Disorders Clinic between September 2006 and October 2007 were invited to participate in the study, together with a group of matched healthy controls. Patients and controls were submitted to comprehensive neuropsychological assessment. MRI was performed in all patients, and abnormalities (high-intensity signal, low-intensity signal and atrophy) were semi-quantitatively rated. Performance of patients and controls in each cognitive test was compared, and correlations between cognitive scores and MRI changes were investigated within the patients' group. RESULTS: Twenty patients with WD (11 men) and 20 controls (nine men) were evaluated. Mean age in the WD and control groups was 30.05 ± 7.25 and 32.15 ± 5.37 years, respectively. Mean schooling years were 11.15 ± 3.73 among WD cases and 10.08 ± 2.62 among controls. Patients with WD performed significantly worse than controls in the Mini-Mental State Examination, Dementia Rating Scale, phonemic verbal fluency (FAS), verb generation, digit span forward, Stroop test, Frontal Assessment Battery and in the Brief Cognitive Screening Battery. A significant correlation emerged between global cognitive impairment and MRI scale (r = 0.535), being higher for high-intensity signal plus atrophy (r = 0.718). CONCLUSION: Patients with WD presented cognitive impairment, especially in executive functions, with good correlation between cognitive abnormalities and MRI changes.

Chronic stress is associated with high cortisol levels and emotional coping mechanisms in amnestic mild cognitive impairment.

BACKGROUND/AIMS: To investigate the association between cortisol levels, chronic stress and coping in subjects with amnestic-type mild cognitive impairment (aMCI). METHODS: Cortisol levels were measured using morning saliva samples from 33 individuals with aMCI and from 41 healthy elderly. Chronic stress was evaluated with the Stress Symptoms List (SSL), whereas coping strategies were assessed using the Jalowiec Coping Scale. RESULTS: aMCI subjects with high SSL scores presented higher cortisol levels (p = 0.045). Furthermore, aMCI subjects who employed emotion-focused coping had higher SSL scores (p = 0.023). CONCLUSION: The association between increased cortisol secretion, chronic stress and coping strategies may be modulated by the presence or absence of cognitive impairment, where memory defi- cit awareness constitutes an additional potential factor involved in high stress severity.

Beyond neuropathy in hereditary sensory and autonomic neuropathy type V: cognitive evaluation.

BACKGROUND AND PURPOSE: Hereditary sensory and autonomic neuropathy (HSAN) type V is a very rare disorder. It is characterized by the absence of thermal and mechanical pain perception caused by decreased number of small diameter neurons in peripheral nerves. Recent genetic studies have pointed out the aetiological role of nerve growth factor beta, which is also involved in the development of the autonomic nervous system and cholinergic pathways in the brain. HSAN type V is usually reported not to cause mental retardation or cognitive decline. However, a structured assessment of the cognitive profile of these patients has never been made. METHODS AND RESULTS: We performed a throughout evaluation of four HSAN type V patients and compared their performance with 37 normal individuals. Our patients showed no cognitive deficits, not even mild ones. DISCUSSION AND CONCLUSIONS: Although newer mutations on this and related disorders are continuously described, their clinical characterization has been restricted to the peripheral aspects of these conditions. A broader characterization of this rare disorder may contribute to better understand the mechanisms of the nociceptive and cognitive aspects of pain.

Patterns of cerebral activation during lexical and phonological reading in Portuguese.

According to the concepts of cognitive neuropsychology, there are two principal routes of reading processing: a lexical route, in which global reading of words occurs and a phonological route, responsible for the conversion of the graphemes into their respective phonemes. In the present study, functional magnetic resonance imaging (fMRI) was used to investigate the patterns of cerebral activation in lexical and phonological reading by 13 healthy women with a formal educational level greater than 11 years. Participants were submitted to a silent reading task containing three types of stimuli: real words (irregular and foreign words), nonwords and illegitimate graphic stimuli. An increased number of activated voxels were identified by fMRI in the word reading (lexical processing) than in the nonword reading (phonological processing) task. In word reading, activation was greater than for nonwords in the following areas: superior, middle and inferior frontal gyri, and bilateral superior temporal gyrus, right cerebellum and the left precentral gyrus, as indicated by fMRI. In the reading of nonwords, the activation was predominant in the right cerebellum and in the left superior temporal gyrus. The results of the present study suggest the existence of differences in the patterns of cerebral activation during lexical and phonological reading, with greater involvement of the right hemisphere in reading words than nonwords.

Leptin in Alzheimer's disease.

Alzheimer's disease (AD) is the most common cause of progressive dementia in the elderly population. AD is histologically characterized by accumulation of amyloid-ß protein (Aß) on extracellular plaques and deposition of hyperphosphorylated tau protein in intracellular neurofibrillary tangles. Several studies have shown that obesity may precede dementia and that lifestyle factors play a critical role in the onset of AD. Furthermore, accumulating evidence indicates that obesity is an independent risk factor for developing AD. In this scenario, the understanding of the role of adipose tissue in brain health is essential to clarify the establishment of demential processes. The objective of this work was to review studies regarding leptin, an anorexigenic peptide hormone synthesized in adipocytes, in the context of dementia. Some authors proposed that leptin evaluation might be a better predictor of dementia than traditional anthropometric measures. Leptin, once established as a biomarker, could enhance the understanding of late-onset AD risk over the life course, as well as the clinical progression of prodromal state to manifested AD. Other studies have proposed that leptin presents neuroprotective activities, which could be explained by inhibiting the amyloidogenic process, reducing the levels of tau protein phosphorylation and improving the cognitive function.

Magnetic resonance imaging in intracranial paracoccidioidomycosis.

Paracoccidioidomycosis is a systemic mycosis, endemic in South and Central America, that affects the central nervous system (CNS) in almost 10% of patients. Neurological involvement includes two different clinical forms: meningeal and granulomatous, also known as the pseudotumor form. Five patients with biopsy-proved systemic paracoccidioidomycosis and neurological complaints were studied by magnetic resonance imaging. CNS involvement was detected in all patients in the form of multiple round or lobulated lesions, predominantly hypointense on T2-weighted images and ring or nodular enhancement on post-gadolinium T1-weighted images. The lesions were distributed diffusely, with a slight predominance in the supratentorial compartment, although infratentorial lesions were also observed, mainly in the cerebellum. Hypointense lesions on T2-weighted images persisted in all 3 patients reexamined after treatment, whereas enhancing lesions on post-gadolinium T1-weighted images turned isointense in 2 patients. Magnetic resonance imaging is a sensitive method in documenting CNS paracoccidioidomycosis, most frequently as supratentorial and infratentorial multiple, round or lobulated hypointense lesions on T2-weighted images.

Brain metastasis of a malignant fibrous histiocytoma of the lung.

Sarcomas metastatic to the brain as well as primary malignant fibrous histiocytomas of the lung are very unusual tumors. The authors report a case of a 52-year-old man who presented with neurological symptoms due to cerebral metastasis of a malignant fibrous histiocytoma of the lung and provide a review of the literature on the subject.

Unexpected reading dissociation in a Brazilian "nisei" with crossed aphasia.

There is an increased interest in reading impairments in the Japanese language, due to its particular writing system which includes two different scripts, Kanji (logograms) and Kana (phonograms). Reading dissociations between Kanji and Kana have been described, showing that each system is processed differently by the cerebral hemispheres. We describe the case of a 68 year old Brazilian "nisei" (i.e. born from Japanese parents) who had knowledge of both Japanese and Portuguese. He presented an ischemic stroke affecting the right hemisphere and subsequently developed a Broca's aphasia and an unexpected reading dissociation, with an impairment in Kana reading comprehension and a good performance in Kanji and in Portuguese. These findings suggest that the patient's right and left hemispheres have assumed opposite roles not only for oral but also for written language decodification.

Encoding process in delayed recall impairment and rate of forgetting in Alzheimer's disease.

OBJECTIVE: To assess the role of impaired encoding in learning and in delayed recall disturbances, and to evaluate the rate of forgetting in AD. METHOD: Fifteen AD patients with mild or moderate dementia and 15 normal matched controls were assessed with the Buschke Selective Reminding Test. Delayed recall was evaluated after 30 minutes and after 24 hours. RESULTS: AD patients had a poorer performance across the six trials of the learning phase as well as in both delayed recall evaluations, with no difference between recall at 30 minutes and at 24 hours. CONCLUSION: Performance in the learning phase was as specific and almost as sensitive as the performance in delayed recall for AD diagnosis. Encoding impairment was responsible for poorer learning and rapid displacement of previous learned material in the AD group. Finally, we did not find a higher rate of forgetting in AD patients.

[Clinical characterization of vascular dementia: retrospective evaluation of an outpatient sample]. / Caracterização clínica da demência vascular: avaliação retrospectiva de uma amostra de pacientes ambulatoriais.

OBJECTIVE: to analyze the clinical features and associated morbidity in a group of patients with vascular dementia (VD). METHODS: we retrospectively evaluated 25 patients with diagnosis of VD, based on the State of California Alzheimers Disease Diagnostic and Treatment Centers (ADDTC) criteria. Clinical and neuroimaging data and laboratory test results were obtained for the characterization of the sample. RESULTS: the mean age was 68.7 +/- 14.6 years (64.0% men), with mean educational level of 5.2 +/- 4.4 years. Sudden onset of symptoms was observed in 48.0% of patients and stepwise deteriorating and fluctuating courses being observed in 4.0% and 16% respectively. Focal neurologic deficits were the first symptom in 48.0%, with focal deficits being observed in 80% on examination. The main morbidity were: hypertension (92.0%); hypercholesterolemia (64.0%); coronary heart disease (40.0%); smoking (40.0%); hypertriglyceridemia (36.0%); diabetes mellitus (32.0%); Chagas' disease (8.0%). CONCLUSIONS: we observed strong association between VD and hypertension and hypercholesterolemia. The observation of two patients presenting Chagas' disease suggests that this endemic condition may be considered a possible regional risk factor.

Involuntary hand levitation associated with parietal damage: another alien hand syndrome.

The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.

Chorea as a sign of systemic lupus erythematosus activity. Case report.

Chorea may occur as a neurological manifestation of systemic lupus erythematosus and is often associated with detection of antiphospholipid antibodies. No evidence of chorea as a sign of lupus activity has been established. We describe a patient with systemic lupus erythematosus associated with antiphospholipid antibodies who developed chorea, which has been considered a sign of lupus activity.

Primary progressive aphasia: analisys of 16 cases.

Primary progressive aphasia (PPA) is an intriguing syndrome, showing some peculiar aspects that differentiate it from classical aphasic pictures caused by focal cerebral lesions or dementia. The slow and progressive deterioration of language occurring in these cases provides an interesting model to better understand the mechanisms involved in the linguistic process. We describe clinical and neuroimaging aspects found in 16 cases of PPA. Our patients underwent language and neuropsychological evaluation, magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT). We observed a clear distinction in oral expression patterns; patients were classified as fluent and nonfluent. Anomia was the earliest and most evident symptom in both groups. Neuroimaging pointed to SPECT as a valuable instrument in guiding the differential diagnosis, as well as in making useful clinical and anatomical correlations. This report and a comparison to literature are an attempt to contribute to a better understanding of PPA.

Brain SPECT in dementia. A clinical-scintigraphic correlation.

The aim of this study was to compare the accuracy of computed tomography (CT) and single photon emission computerized tomography (SPECT) in the diagnosis of dementia. Fifty-two patients with clinical diagnosis of dementia and 11 controls were studied. The scans were interpreted by one experienced neuroradiologist and one nuclear radiologist, both blinded to the clinical data. In the diagnosis of dementia, CT and SPECT showed equal sensitivity (82.7%) and statistically similar specificity (63.8 and 81.8%, respectively). The specificity of SPECT in diagnosing Alzheimer's disease (100%) was statistically superior to CT (69%). However, both methods showed similar sensitivity in detecting Alzheimer's disease. In conclusion, SPECT and CT showed similar accuracy in the diagnosis of dementia. The quite high specificity of SPECT in Alzheimer's disease may be useful for confirming that diagnosis, particularly for patients with presenile onset of the disease.

[Neuropsychological tests of simple application for diagnosing dementia]. / Testes neuropsicológicos de aplicação simples para o diagnóstico de demência.

Thirty patients with dementia defined by DSM-III-R criteria (Alzheimer's disease (22), vascular dementia (3), Parkinson's disease, frontal lobe dementia, possible diffuse Lewy body dementia, normal pressure hydrocephalus and uncertain diagnosis), with scores below 24 points in the Mini-Mental Status Examination and more than 4 years of education were submitted to a neuropsychological evaluation. The scores in the neuropsychological tests were compared to those obtained by thirty normal volunteers paired for age, sex and education. Sensitivity, specificity and accuracy of the tests in the distinction of demented and normal volunteers were determined. The accuracies were calculated using ROC curves. Blessed's information-memory-concentration test showed greatest accuracy, followed by copy of simple figures, delayed memory of 10 figures (after 5 minutes), recognition of 10 figures and verbal fluency test (animals). A linear discriminant function, composed by 6 tests: visual perception, incidental memory, delayed memory (after 5 minutes), drawing of a clock, verbal fluency (animals) and calculation tests, was able to discriminate all controls from patients and only one patient was wrongly classified as normal control. These tests were chosen because they can be applied in less than 10 minutes and are very easy to interpret. This discriminant function must be applied in another group of patients and controls in order to demonstrate its value. When associated to the MMSE it may be useful to discriminate patients with dementia from normal people in epidemiological studies.

Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17.

OBJECTIVE: To compare the clinical features of a familial prion disease with those of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). BACKGROUND: Prion diseases are not usually considered in the differential diagnosis of FTDP-17, since familial Creutzfeldt-Jakob disease (CJD), the most common inherited prion disease, often manifests as a rapidly progressive dementia. Conversely, FTDP-17 usually has an insidious onset in the fifth decade, with abnormal behavior and parkinsonian features. METHOD: We present the clinical features of 12 patients from a family with CJD associated with a point mutation at codon 183 of the prion protein gene. RESULTS: The mean age at onset was 44.0 +/- 3.7; the duration of the symptoms until death ranged from two to nine years. Behavioral disturbances were the predominant presenting symptoms. Nine patients were first seen by psychiatrists. Eight patients manifested parkinsonian signs. CONCLUSION: These clinical features bear a considerable resemblance to those described in FTDP-17.

Neurological examination in obsessive-compulsive disorder.

Recent studies suggest the occurrence of a neurological dysfunction in Obsessive-Compulsive Disorder (OCD). The purpose of the present study was to verify the clinical value of a neurological evaluation in patients with the disease. We submitted 15 patients with OCD (five of whom were under clomipramine) and 15 controls in a detailed neurological examination, including assessment of the neurological soft-signs. Eleven patients (73.3 percent) and four controls (26.7 percent) presented abnormalities on examination. The main findings among the patients were: palmomental reflex (six cases); mirror movements (five cases); agraphestesia and dysdiadochokinesia (three cases). Three out of the four patients who had a normal examination were on clomipramine. Palmomental reflex was the main finding among the controls. These results, although preliminary, stress the interest and usefulness of performing a detailed neurological examination in OCD.

[Thrombolytic treatment in pulmonary thromboembolism in the postoperative period following heart surgery complicated by ischemic cerebrovascular attack]. / Tratamento trombolítico no tromboembolismo pulmonar em pós-operatório de cirurgia cardíaca complicado por acidente vascular cerebral isquêmico.

A 69 years old man with pulmonary embolism early after coronary bypass surgery complicated by ischemic stroke received thrombolytic therapy. Reperfusion of the pulmonary artery was achieved. Conversion of the ischemic stroke to hemorrhagic infarction was observed at the CT-Scan without neurological impairment.

Spectrum of cognitive impairment in neurocysticercosis: differences according to disease phase.

OBJECTIVES: Cognitive decline related to neurocysticercosis (NC) remains poorly characterized and underdiagnosed. In a cross-sectional study with a prospective phase, we evaluated cognitive decline in patients with strictly calcified form (C-NC), the epidemiologically largest subgroup of NC, and investigated whether there is a spectrum of cognitive abnormalities in the disease. METHODS: Forty treatment-naive patients with C-NC aged 37.6 ± 11.3 years and fulfilling criteria for definitive C-NC were submitted to a comprehensive cognitive and functional evaluation and were compared with 40 patients with active NC (A-NC) and 40 healthy controls (HC) matched for age and education. Patients with dementia were reassessed after 24 months. RESULTS: Patients with C-NC presented 9.4 ± 3.1 altered test scores out of the 30 from the cognitive battery when compared to HC. No patient with C-NC had dementia and 10 patients (25%) presented cognitive impairment-no dementia (CIND). The A-NC group had 5 patients (12.5%) with dementia and 11 patients (27.5%) with CIND. On follow-up, 3 out of 5 patients with A-NC with dementia previously still presented cystic lesions with scolex on MRI and still had dementia. One patient died and the remaining patient no longer fulfilled criteria for either dementia or CIND, presenting exclusively calcified lesions on neuroimaging. CONCLUSIONS: Independently of its phase, NC leads to a spectrum of cognitive abnormalities, ranging from impairment in a single domain, to CIND and, occasionally, to dementia. These findings are more conspicuous during active vesicular phase and less prominent in calcified stages.