Gesture comprehension, knowledge and production in Alzheimer's disease.

BACKGROUND AND PURPOSE: Although apraxia is a typical consequence of Alzheimer's disease (AD), the profile of apraxic impairments is still subject to debate. Here, we analysed apraxia components in patients with AD with mild-to-moderate or moderately severe dementia. METHODS: Thirty-one patients were included. We first evaluated simple gestures, that is, the imitation of finger and hand configurations, symbolic gestures (recognition, production on verbal command and imitation), pantomimes (recognition, production on verbal command, imitation and production with the object), general knowledge and complex gestures (tool-object association, function-tool association, production of complex actions and knowledge about action sequences). Tests for dementia (Mini Mental State Examination and the Dementia Rating Scale), language disorders, visual agnosia and executive function were also administered. RESULTS: Compared with controls, patients showed significant difficulties (P ≤ 0.01) in subtests relating to simple gestures (except for the recognition and imitation of symbolic gestures). General knowledge about tools, objects and action sequences was less severely impaired. Performance was frequently correlated with the severity of dementia. Multiple-case analyses revealed that (i) the frequency of apraxia depended on the definition used, (ii) ideomotor apraxia was more frequent than ideational apraxia, (iii) conceptual difficulties were slightly more frequent than production difficulties in the early stage of AD and (iv) difficulties in gesture recognition were frequent (especially for pantomimes). CONCLUSION: Patients with AD can clearly show gesture apraxia from the mild-moderate stage of dementia onwards. Recognition and imitation disorders are relatively frequent (especially for pantomimes). We did not find conceptual difficulties to be the main problem in early-stage AD.

[Primitive leptomeningeal malignant melanoma: a rare etiology of pachymeningitis and leptomeningitis]. / Mélanome malin leptoméningé primitif : une étiologie rare de pachy- et leptoméningite.

INTRODUCTION: Leptomeningitis and pachymeningitis are known to occur consecutive to many causes. OBSERVATION: We report the case of a 24-year-old woman with symptoms of raised intracranial pressure and repeated switching transient hemiparesis. The magnetic resonance imaging (MRI) showed a pachyleptomeningitis. Search for a cause was negative. The pathology examination of meningeal tissue revealed a malignant melanoma, without any sign of cutaneous melanoma, leading to the diagnosis of primary leptomeningeal malignant melanoma. CONCLUSION: A meningeal biopsy can enable the rare diagnosis of primary leptomeningeal malignant melanoma in a patient with unexplained pachyleptomeningitis.

Prediction of pathology in primary progressive language and speech disorders.

OBJECTIVE: Frontotemporal lobar degeneration (FTLD) encompasses a variety of clinicopathologic entities. The antemortem prediction of the underlying pathologic lesions is reputed to be difficult. This study sought to characterize correlations between 1) the different clinical variants of primary progressive language and speech disorders and 2) the pathologic diagnosis. METHODS: The latter was available for 18 patients having been prospectively monitored in the Lille Memory Clinic (France) between 1993 and 2008. RESULTS: The patients were diagnosed with progressive anarthria (n = 5), agrammatic progressive aphasia (n = 6), logopenic progressive aphasia (n = 1), progressive jargon aphasia (n = 2), typical semantic dementia (n = 2), and atypical semantic dementia (n = 2). All patients with progressive anarthria had a tau pathology at postmortem evaluation: progressive supranuclear palsy (n = 2), Pick disease (n = 2), and corticobasal degeneration (n = 1). All patients with agrammatic primary progressive aphasia had TDP-43-positive FTLD (FTLD-TDP). The patients with logopenic progressive aphasia and progressive jargon aphasia had Alzheimer disease. Both cases of typical semantic dementia had FTLD-TDP. The patients with atypical semantic dementia had tau pathologies: argyrophilic grain disease and corticobasal degeneration. CONCLUSIONS: The different anatomic distribution of the pathologic lesions could explain these results: opercular and subcortical regions in tau pathologies with progressive anarthria, the left frontotemporal cortex in TDP-43-positive frontotemporal lobar degeneration (FTLD-TDP) with agrammatic progressive aphasia, the bilateral lateral and anterior temporal cortex in FTLD-TDP or argyrophilic grain disease with semantic dementia, and the left parietotemporal cortex in Alzheimer disease with logopenic progressive aphasia or jargon aphasia. These correlations have to be confirmed in larger series.

[VZV-related myelitis: a pathophysiological hypothesis]. / Myélite à VZV: hypothèses physiopathologiques.

INTRODUCTION: Complications of VZV infection in the central nervous system are multiple. VZV-related myelitis is an uncommon complication of herpes zoster. OBSERVATION: We report the case of a 55-year old man with intercostal herpes zoster who presented a subacute medullar syndrome. MRI demonstrated an extended cervico-thoracic medullar hyperintensity on the T2-weighted images. Cerebrospinal fluid (CSF) analysis showed 100 leukocytes/mm3, 0.94 g/L protein, negative VZV PCR, elevated rate of anti-VZV IgG and no oligoclonal bands. Clinical, biological and radiological presentations were compatible with the diagnosis of VZV-related myelitis with three potential pathophysiological mechanisms: infectious, immune post-infectious, vascular. The course was partially favorable after a 3-day regimen of corticosteroid and 3 weeks of acyclovir infusions. DISCUSSION: Parainfectious myelitis is often the consequence of a viral infection with a post-infectious pathogenesis. Most often, the clinical outcome is good. In this case report, we highlight the VZV vascular tropism and its more severe outcome. CONCLUSION: VZV-related myelitis should be diagnosed early. The combination of aciclovir and corticoids infusions seems to be beneficial.

Angioplasty and stenting for high-grade internal carotid artery stenosis: safety study in 39 selected patients.

BACKGROUND: Carotid angioplasty and stenting (CAS) is sometimes used as an alternative to surgery, despite the lack of evidence for its safety and efficacy. METHOD: Over a 33-month period, 39 consecutive patients with a stenosis >/=70% underwent CAS (4 in a randomized trial and 35 because of contra-indications for surgery). RESULTS: In 5 patients (13%; 95% CI: 3-23), a major complication occurred (3 disabling ischaemic strokes, 1 myocardial infarction, 1 acute interstitial nephropathy). In 7 patients (18%; 95% CI: 6-30), a minor complication occurred (5 transient ischaemic attacks, 1 transient confusional state, 1 non-disabling ischaemic stroke). CONCLUSION: CAS cannot be considered as a routine procedure and should be restricted to high-risk patients unfit for surgery.

Cerebral venous thrombosis 3-year clinical outcome in 55 consecutive patients.

An early diagnosis and heparin therapy have contributed to a decreased mortality in cerebral venous thrombosis (CVT). However, predictors of outcome are difficult to identify, because most studies suffered heterogeneity in diagnostic findings and treatments, retrospective design, and recruitment bias. The aim of this study was to evaluate the clinical outcome in 55 consecutive patients with CVT admitted over a 4-year period. The study population consisted of 42 women and 13 men, with a median age of 39 years (range 16-68). The diagnosis was performed with MRI in 53 patients, and angiography in 2. The outcome was assessed with the modified Rankin scale (mRs). After a median follow-up of 36 months (range: 12-60), 45 patients were independent (mRS 0-2), and 10 were dependent or dead (mRS 3-6). Of 48 survivors, 7 had seizures, 6 motor deficits, 5 visual field defects, 29 headache (migraine in 14, tension headache in 13, other in 2). The logistic regression analysis found focal deficits and cancer at time of diagnosis, as independent predictors of dependence or death at year 3, and isolated intra-cranial hypertension as an independent predictor of survival and independence. Mortality rates are low in the absence of cancer and focal deficits, and more than 80 % of survivors are independent after 3 years. However, 3/4 of survivors have residual symptoms. Therefore, despite a low mortality rate, CVT remains a serious disorder.

[Sarcoidosis and progressive multifocal leukoencephalopathy]. / Sarcoïdose et leucoencéphalopathie multifocale progressive.

A 70-year-old woman treated for sarcoidosis complained of progressive cognitive impairment and gait disability. Magnetic resonance imaging of the brain revealed a nonenhancing lesion in T1-weighted imaging in the left parieto-occipital region and sarcoidosis of the central nervous system was evoked. However, she rapidly deteriorated with posterior and cerebellar extension of the lesions, suggesting of progressive multifocal leukoencephalopathy (PML). DNA of the JC virus (JCV) was detected in the cerebrospinal fluid (CSF) by a polymerase chain reaction. Despite antiviral therapy, she died nine months after the first neurological signs. This case illustrates the possible association between sarcoidosis and PML, and underlines the interest to detect the presence of JCV in the CSF when the diagnosis of neurosarcoidosis appeared uncertain.

[Multiple sclerosis and antiphospholipid antibodies: study of 62 consecutive patients]. / Sclérose en plaques et anticorps antiphospholipides: étude consécutive de 62 patients.

INTRODUCTION: Although multiple sclerosis (MS) and antiphospholipid syndrome (AS) are usually defined by specific criteria that make them distinguishable, in some cases, transition between the two diseases based on clinical and brain imaging findings is not clear. METHODS: Our study included 62 patients (sex ratio F/M = 1.48; mean age 43.4 +/- 23.6 years) with diagnosis of MS according to Poser criteria and 31 control subjects (sex ratio F/M = 9.3, mean age 37 +/- 17 years). We examined the level of antibodies against phospholipids (anticardiolipid, anti beta 2-glycoprotein 1 and antiphosphatidylethanolamine antibodies), antinuclear, anti native DNA, antiprothrombinase antibodies and rheumatoid factor. RESULTS: Antiphospholipid antibodies were found with a significant level (anticardiolipid > 30 UI, anti beta 2-glycoprotein 1 positive) in only five patients (8%) with MS; two others showed an increase in antinuclear antibodies (1/320 degrees and 1/1280 degrees). CONCLUSION: In contrast with data recently reported, this study failed to find a significant level of antiphospholipid antibodies in MS. This result argues for the existence of different pathogenic mechanisms in MS and AS.