RÉSUMÉ
The increasing prevalence of Alzheimer's dementia (AD) and limited resources in outpatient care have encouraged the distribution of cognitive screening tests, in spite of their frequently unsatisfying accuracy regarding the differentiation between incipient AD, depression and age-associated memory impairment. 8 patients with probable AD and 17 controls completed a neuropsychological follow-up two years after initial examination. Beside four screening tests a memory based testing-the-limits (TtL) paradigm as well as the German version of the California Verbal Learning Test were administered. Based on hierarchical cluster analysis we could demonstrate that only well elaborated tests, such as a plasticity based TtL paradigm, did classify AD-patients correctly. The findings confirm reservations against cognitive screening procedures in detecting dementia and suggest that dynamic test strategies offer a powerful diagnostic alternative to traditional status-oriented tests.
Sujet(s)
Cognition/physiologie , Tests neuropsychologiques/normes , Sujet âgé , Sujet âgé de 80 ans ou plus , Maladie d'Alzheimer/diagnostic , Maladie d'Alzheimer/psychologie , Analyse de regroupements , Interprétation statistique de données , Dépression/diagnostic , Dépression/psychologie , Femelle , Humains , Mâle , Mémoire/physiologie , Adulte d'âge moyen , Apprentissage verbalRÉSUMÉ
Since valosin-containing protein mutations were reported as a cause of hereditary inclusion body myositis associated with Paget's disease of the bone and frontotemporal dementia, many new mutations have been described in the last decade. We report on a 46-year-old German male with a progressive tetraparesis and autosomal dominant inheritance pattern. Echocardiography revealed a beginning dilated cardiomyopathy and laboratory analyses showed increased alkaline phosphatase. Decreased verbal memory and an impairment of concept building were observed on neuropsychological examination. Muscle biopsy demonstrated a myopathic pattern, rimmed vacuoles, CD8+ T-cell infiltrates and positive MHC1-muscle fibres. We found a heterozygote mutation in exon 5 of the valosin-containing protein gene (c.464G > T p.Arg155Leu), which until now has been described only in an Australian family. We describe here the first German case with the above-mentioned mutation causing inclusion-body myositis associated with Paget's disease of the bone and fronto-temporal dementia. Here, we recommend regular controls of cardiac and respiratory functions.
Sujet(s)
Démence frontotemporale/complications , Démence frontotemporale/physiopathologie , Coeur/physiopathologie , Myosite à inclusions/complications , Myosite à inclusions/physiopathologie , Maladie de Paget des os/complications , Maladie de Paget des os/physiopathologie , Muscles respiratoires/physiopathologie , Âge de début , Cardiomyopathie dilatée/complications , Cardiomyopathie dilatée/imagerie diagnostique , Échocardiographie , Démence frontotemporale/génétique , Humains , Mâle , Adulte d'âge moyen , Myosite à inclusions/génétique , Tests neuropsychologiques , Maladie de Paget des os/génétique , Pedigree , Tétraplégie/étiologie , Tétraplégie/génétique , Tétraplégie/physiopathologie , Muscles respiratoires/anatomopathologieRÉSUMÉ
BACKGROUND: Hippocampal sulcal cavities (HSC) have been speculated to contribute to a higher vulnerability of memory pathways and might be a possible etiological factor in transient global amnesia (TGA). Therefore, we evaluated the influence of HSC on cognitive long-term outcome in TGA-patients. METHODS: Fourteen otherwise healthy patients with the clinical syndrome of TGA in their history underwent a high-resolution magnetic resolution imaging and a comprehensive neuropsychological test battery. The neuropsychological control group consisted of 15 healthy subjects and was balanced for age, sex and other risk factors as well as intellectual and social status. RESULTS: Magnetic resolution imaging and neuropsychological testing have been performed 1128 days (median) after the TGA. HSC have been detected in nine of the 14 patients and have been bilateral in eight of them. There were no differences in cognitive performance in patients with and without HSC as well as compared to healthy subjects. Even in patients with greater lesion load, only a slight visuospatial deficit was found. CONCLUSIONS: Although an increased incidence of HSCs is detected in TGA patients, cavities are not obligatorily in TGA. Moreover, even patients with hippocampal cavities achieve a full neuropsychological recovery independent of the frequency and size of the hippocampal lesions.
Sujet(s)
Amnésie globale transitoire/anatomopathologie , Troubles de la cognition/anatomopathologie , Hippocampe/malformations , Malformations du système nerveux/anatomopathologie , Sujet âgé , Amnésie globale transitoire/étiologie , Amnésie globale transitoire/physiopathologie , Cognition/physiologie , Troubles de la cognition/étiologie , Troubles de la cognition/physiopathologie , Femelle , Humains , Imagerie par résonance magnétique/méthodes , Mâle , Mémoire/physiologie , Adulte d'âge moyen , Malformations du système nerveux/complications , Malformations du système nerveux/physiopathologie , Tests neuropsychologiques/normesRÉSUMÉ
Posterior cortical atrophy (PCA) is a neurodegenerative disease which leads to a dementing syndrome that involves an irreversible impairment of higher visual and spatial functions. Memory and language functions generally tend to be preserved better than in other types of dementia including Alzheimer's disease. Here we report a case of PCA, which initially was diagnosed and treated for about a year as a major depressive episode. While most patients initially present with neurologic symptoms, in some PCA cases secondary manifestations, such as e. g. affective symptoms, might appear before the onset of overt cognitive dysfunction. In some cases, this might lead to a diagnostic delay of this neurodegenerative disease.
Sujet(s)
Encéphalopathies/anatomopathologie , Cortex cérébral/anatomopathologie , Trouble dépressif/étiologie , Trouble dépressif/psychologie , Maladies neurodégénératives/étiologie , Maladies neurodégénératives/psychologie , Affect , Antidépresseurs de seconde génération/usage thérapeutique , Atrophie , Encéphalopathies/imagerie diagnostique , Cortex cérébral/imagerie diagnostique , Troubles de la cognition/étiologie , Troubles de la cognition/psychologie , Cyclohexanols/usage thérapeutique , Trouble dépressif/traitement médicamenteux , Électroencéphalographie , Potentiels évoqués/physiologie , Humains , Imagerie par résonance magnétique , Mâle , Adulte d'âge moyen , Maladies neurodégénératives/anatomopathologie , Tomographie par émission de positons , Chlorhydrate de venlafaxineRÉSUMÉ
BACKGROUND AND PURPOSE: A novel presenilin1 (PSEN1) mutation associated with dementia and spastic paraplegia in a family with five affected individuals is described. The index patient was a 35-year-old man presenting with cognitive decline, behavioural symptoms, dysarthria, and gait disorder due to spasticity. METHODS AND RESULTS: Genetic analysis revealed a missense mutation Gln223Arg in exon 7. Initial CSF analysis revealed drastically decreased Abeta42 level despite marginally decreased FDG metabolism. CONCLUSION: Cerebrospinal fluid biomarker analysis might point towards genetic analysis of PSEN1 in patients with positive family history and age of onset below 60 years.
Sujet(s)
Maladie d'Alzheimer/génétique , Peptides bêta-amyloïdes/liquide cérébrospinal , Dysarthrie/génétique , Mutation faux-sens , Paraparésie spastique/génétique , Préséniline-1/génétique , Adulte , Âge de début , Maladie d'Alzheimer/liquide cérébrospinal , Maladie d'Alzheimer/anatomopathologie , Encéphale/imagerie diagnostique , Encéphale/métabolisme , Encéphale/anatomopathologie , Dysarthrie/liquide cérébrospinal , Dysarthrie/anatomopathologie , Famille , Femelle , Fluorodésoxyglucose F18 , Humains , Imagerie par résonance magnétique , Mâle , Paraparésie spastique/liquide cérébrospinal , Paraparésie spastique/anatomopathologie , Pedigree , Tomographie par émission de positonsRÉSUMÉ
Twenty-six Austrian, Dutch, German, and Swiss epilepsy centers were asked to report on use of the Wada test (intracarotid amobarbital procedure, IAP) from 2000 to 2005 and to give their opinion regarding its role in the presurgical diagnosis of epilepsy. Sixteen of the 23 centers providing information had performed 1421 Wada tests, predominantly the classic bilateral procedure (73%). A slight nonsignificant decrease over time in Wada test frequency, despite slightly increasing numbers of resective procedures, could be observed. Complication rates were relatively low (1.09%; 0.36% with permanent deficit). Test protocols were similar even though no universal standard protocol exists. Clinicians rated the Wada test as having good reliability and validity for language determination, whereas they questioned its reliability and validity for memory lateralization. Several noninvasive functional imaging techniques are already in use. However, clinicians currently do not want to rely solely on noninvasive functional imaging in all patients.
Sujet(s)
Épilepsie/physiopathologie , Langage , Mémoire/physiologie , Tests neuropsychologiques/statistiques et données numériques , Autriche , Allemagne , Humains , Études multicentriques comme sujet , Pays-Bas , SuisseRÉSUMÉ
Transient global amnesia (TGA) is a clinical syndrome of unknown etiology characterized by sudden onset anterograde amnesia, which was thought to resolve completely. However, some authors have also suggested permanent memory impairment. It is unclear whether these results reflect a true persistent damage or a simply too short assessment interval in the context of a prolonged recovery phase after TGA. To evaluate the cognitive long-term outcome, 16 patients who had suffered from TGA at a mean of 3 years before and 15 healthy controls underwent a comprehensive neuropsychological test battery. No significant differences between patients' and controls' cognitive performance were found, irrespectively of the analyzed neuropsychological domain. Therefore we hypothesize that TGA usually does not cause persistent cognitive deficits due to a generally transient and prognostic benign character.
Sujet(s)
Amnésie/complications , Troubles de la cognition/étiologie , Récupération fonctionnelle/physiologie , Adulte , Sujet âgé , Attention/physiologie , Femelle , Humains , Mâle , Mémoire/physiologie , Adulte d'âge moyen , Tests neuropsychologiques/statistiques et données numériques , Résolution de problème/physiologieRÉSUMÉ
Treatment with high-dose corticosteroids over 3-5 days reduces the duration and severity of relapses in patients with multiple sclerosis. Since the benefit of this treatment appears to be dose-dependent, application of ultrahigh steroid doses becomes important. On the other hand, data from basic research and clinical studies indicate potential side effects on mnestic functions. Three recently published studies show that treatment with pulsed corticosteroids induces reversible impaired memory. The effects were independent of the dose administered.
Sujet(s)
Troubles de la cognition/induit chimiquement , Cortisone/administration et posologie , Cortisone/effets indésirables , Troubles de la mémoire/induit chimiquement , Sclérose en plaques/traitement médicamenteux , Anti-inflammatoires/administration et posologie , Anti-inflammatoires/effets indésirables , Cognition/effets des médicaments et des substances chimiques , Troubles de la cognition/prévention et contrôle , Relation dose-effet des médicaments , Humains , Troubles de la mémoire/prévention et contrôle , Sclérose en plaques/complications , Appréciation des risques/méthodes , Facteurs de risqueRÉSUMÉ
Mirror movements (MM), involuntary movements of homologous muscles opposite to unilateral limb activity, decrease in the course of motor development, but may reappear in adults after brain damage. To better characterize this type of acquired MM, grip forces of 36 healthy subjects and 35 patients suffering from focal brain lesions were compared. Holding force transducers in a pinch grip between thumb and index fingers of each hand, subjects had to repeatedly change the grip force in one hand, whilst the other (mirror) hand just had to prevent the manipulandum from dropping. In a second task, force changes had to be produced either in a symmetric or in an antiparallel manner. No significant group differences between patients and controls in the extent of coupling were found. During unimanual squeezing, only six patients had a co-activation in the pathologic range. Whilst performing bimanual asymmetric grip force changes, pathologic coupling was seen only in five patients. No association was found between the presence of pathologic MM and clinical or neuroanatomical features. Brain lesions in adults seem only rarely to be associated with pathologic MM. They might be non-specific and not related to the characteristics of the lesion.
Sujet(s)
Lésions encéphaliques/physiopathologie , Aptitudes motrices/physiologie , Troubles de la motricité/physiopathologie , Adolescent , Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Lésions encéphaliques/complications , Femelle , Force de la main/physiologie , Humains , Mâle , Adulte d'âge moyen , Troubles de la motricité/étiologieRÉSUMÉ
Thirty patients with multiple sclerosis were randomized to 500 or 2,000 mg of methylprednisolone (MP) over 5 days. They were prospectively studied neuropsychologically before and at days 6 and 60 after onset of the therapy, using a double-blind study design. Patients showed selective deterioration of declarative memory retrieval at day 6, which was fully reversible at day 60. Although the sample size was small, these effects were independent of the administered MP dose.
Sujet(s)
Troubles de la mémoire/induit chimiquement , Méthylprednisolone/effets indésirables , Sclérose en plaques/traitement médicamenteux , Adolescent , Adulte , Anti-inflammatoires/administration et posologie , Anti-inflammatoires/effets indésirables , Relation dose-effet des médicaments , Méthode en double aveugle , Calendrier d'administration des médicaments , Femelle , Hippocampe/effets des médicaments et des substances chimiques , Hippocampe/physiopathologie , Humains , Mâle , Troubles de la mémoire/physiopathologie , Méthylprednisolone/administration et posologie , Adulte d'âge moyen , Études prospectivesRÉSUMÉ
We examined an obese 58-year-old patient with a bilateral posterior hypothalamic lesion of unknown etiology. A 24-hour polysomnography revealed a markedly increased total sleep time (17.6 h). During daytime, only 3 continuous wake phases occurred. REM periods occurred only between 5 p.m. and 6 a.m. We conclude from our results that, similar to the results from animal experiments, the posterior hypothalamus in humans plays a critical role in the maintenance of wakefulness.
Sujet(s)
Troubles du sommeil par somnolence excessive/diagnostic , Hypothalamus postérieur/anatomopathologie , Imagerie par résonance magnétique , Réaction antigène-anticorps , Hémogramme , Température du corps , Liquide cérébrospinal/cytologie , Troubles du sommeil par somnolence excessive/physiopathologie , Troubles du sommeil par somnolence excessive/psychologie , Femelle , Hormones/sang , Humains , Adulte d'âge moyen , Tests neuropsychologiques , PolysomnographieRÉSUMÉ
Cognitive impairment is a common phenomenon in children with neurofibromatosis type 1 (NF1), but only little is known about its nature and frequency in adult NF1-patients. Using a comprehensive psychometric test battery, we investigated 20 patients with NF1 and 20 age and gender matched control subjects without neurological diseases. Results showed slightly lowered test scores in patients compared with controls but no specific intellectual impairment. On a computerized test of selective attention, the NF1-group had significant slower reaction times. Also, three out of four memory tests and a test of visuoconstructive abilities showed poorer test results in the NF1-patients. Executive functions however were not affected. The findings agreed well with the test profile in NF1-children and supported the idea of a continuum between childhood and adulthood. Observations are discussed in the context of studies investigating the association of cognitive deficits with either intracranial lesions or alterations in the neurofibromin expression.
Sujet(s)
Troubles de la cognition/étiologie , Troubles de la cognition/psychologie , Neurofibromatose de type 1/complications , Neurofibromatose de type 1/psychologie , Adulte , Vieillissement/psychologie , Femelle , Humains , Tests d'intelligence , Apprentissage/physiologie , Mâle , Mémoire/physiologie , Adulte d'âge moyen , Neurofibromine-1/métabolisme , Tests neuropsychologiques , Temps de réaction/physiologie , Perception de l'espace/physiologie , Perception visuelle/physiologie , Tests d'association verbaleRÉSUMÉ
Cognitive impairment in neurofibromatosis type I (NF1) has only recently attracted interest. In addition to previous studies in children, our own investigation of 20 patients confirms the slightly lowered psychometric test scores also for adults with NF1. Molecular manipulation of the neurofibromin gene leads to learning disorders in the mouse model ("cognitive neurogenetics"). It is not just faulty brain development that underlies these findings: neurofibromin plays a role in signal transduction cascades that are active during learning and memory throughout the life span. Thus, it appears possible to cure the cognitive defects at least in the mouse ("cognitive enhancement"). In man, an early diagnosis of NF1 is presently essential in order to provide specific remedial education for children affected by the learning disorder of neurofibromatosis type I.
Sujet(s)
Troubles de la cognition/génétique , Intelligence/génétique , Neurofibromatose de type 1/génétique , Tests neuropsychologiques , Adulte , Animaux , Enfant , Chromosomes humains de la paire 22 , Troubles de la cognition/diagnostic , Femelle , Humains , Mâle , Souris , Mutants neurologiques de souris , Adulte d'âge moyen , Protéines G monomériques/génétique , Mutation/génétique , Neurofibromatose de type 1/diagnostic , Neurofibromine-1/génétique , Transduction du signal/génétiqueRÉSUMÉ
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a microangiopathic syndrome. Although the defective gene has been identified, genetic analysis may be effort some due to its large size and various mutations. Providing a reliable diagnostic marker would therefore be helpful. Electron microscopy has revealed characteristic electron-dense granular deposits in the basal lamina of vessels of patients with CADASIL. We investigated the sensitivity of skin and muscle biopsies for diagnosing CADASIL. We examined 30 family members of three unrelated German families affected by CADASIL. In 14 of the 21 affected individuals we performed skin and muscle biopsies; two patients were clinically asymptomatic. Under electron microscopy all muscle and skin biopsy specimens showed patches of granular and electron-dense material in the basal layer of both arterioles and capillaries. These findings confirm that general microangiopathy is a typical feature of this syndrome and is present in the early phase of the disease with or without clinical manifestation. Thus, as electron microscopy of skin biopsy specimens can establish the diagnosis of CADASIL with high certainty, it may be considered the method of first choice.
Sujet(s)
Démence par infarctus multiples/diagnostic , Leucoencéphalopathie multifocale progressive/diagnostic , Muscles squelettiques/anatomopathologie , Peau/anatomopathologie , Adulte , Biopsie , Démence par infarctus multiples/génétique , Démence par infarctus multiples/anatomopathologie , Diagnostic différentiel , Femelle , Humains , Mâle , Adulte d'âge moyen , Pedigree , Sensibilité et spécificité , SyndromeRÉSUMÉ
OBJECTIVE: To study correlations between total lesion load on brain MRI and clinical features, and to evaluate the influence of demographic variables on quantitative MRI variables in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). BACKGROUND: CADASIL is a hereditary form of small-vessel disease caused by mutations within the Notch3 gene. MRI abnormalities have been found both in asymptomatic and symptomatic CADASIL individuals. METHODS: Quantitative measurements on cerebral MRI were performed in 64 CADASIL individuals. MRI lesions were quantified using a semi-automated segmentation technique based on local thresholds. RESULTS: MRI total lesion volume correlated significantly with disability (Rankin Scale) on both T1- and proton density (PD)-weighted images. There was a significant inverse correlation between total lesion volume and overall cognitive performance as determined by the Mini-Mental State Examination. Age but not sex was correlated with lesion load both on T1- and PD-weighted images. There was no detectable influence of the Notch3 genotype on quantitative MRI variables. CONCLUSIONS: This study demonstrates correlations between MRI lesion volume and clinical characteristics in CADASIL. Longitudinal studies are now warranted to investigate whether quantitative MRI could be used as an adjunct outcome measure in future therapeutic trials in CADASIL.
Sujet(s)
Artériopathies cérébrales/anatomopathologie , Infarctus cérébral/anatomopathologie , Leucoencéphalopathie multifocale progressive/anatomopathologie , Imagerie par résonance magnétique/méthodes , Adulte , Sujet âgé , Encéphale/anatomopathologie , Encéphale/physiopathologie , Artériopathies cérébrales/psychologie , Infarctus cérébral/psychologie , Cognition/physiologie , Évaluation de l'invalidité , Femelle , Humains , Leucoencéphalopathie multifocale progressive/psychologie , Mâle , Adulte d'âge moyenRÉSUMÉ
BACKGROUND AND PURPOSE: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an arteriopathy related to a genetic defect of the notch 3 gene on chromosome 19. The purpose of this study was to evaluate lesion distribution and volume using MR imaging and to correlate the lesion volume with the neurologic and neuropsychological findings. METHODS: Twenty members of two families (14 with CADASIL as determined by linkage analysis, six healthy) were studied with MR imaging. Two observers evaluated the MR findings semiquantitatively and quantitatively. MR results were then correlated with neurologic and neuropsychological findings. RESULTS: A typical pattern of lesion distribution in patients with CADASIL was found: the frontal lobe was the site with the highest lesion load, followed by the temporal lobe and the insula. The total lesion volume on T1-weighted MR images correlated significantly with the degree of disability and the degree of impairment in neuropsychological functions (including attention, memory, and conceptual and visuospatial functions). CONCLUSION: In CADASIL patients, a common pattern of cerebral lesion distribution is found. The total T1 lesion volume is an important parameter to correlate with disability, as it may prove to be helpful in predicting the natural history of the disease.
Sujet(s)
Encéphalopathies/diagnostic , Encéphalopathies/génétique , Encéphale/anatomopathologie , Imagerie par résonance magnétique , Examen neurologique , Tests neuropsychologiques , Adulte , Sujet âgé , Encéphalopathies/psychologie , Infarctus cérébral/diagnostic , Infarctus cérébral/génétique , Infarctus cérébral/psychologie , Angiopathies intracrâniennes/diagnostic , Angiopathies intracrâniennes/génétique , Angiopathies intracrâniennes/psychologie , Démence par infarctus multiples/génétique , Femelle , Humains , Mâle , Adulte d'âge moyenRÉSUMÉ
We present a non-invasive epilepsy surgery protocol, which includes EEG-video-monitoring, magnetic resonance imaging (MRI), interictal positron emission tomography (PET) and ictal single photon emission computerized tomography (SPECT). According to this non-invasive protocol 50 of 173 patients with medically intractable focal epilepsy underwent resective surgery. The localization of the epileptogenic zone was based on the congruence of the localizing results of EEG-video-monitoring, MRI, interictal PET and ictal SPECT. 46 (92%) of the patients had temporal and 4 (8%) had extratemporal epilepsies. 78% (n = 39) of all patients operated according to our non-invasive protocol were postoperatively completely or almost seizure free. Extramesiotemporal resections could be carried out without invasive EEG-recording if the epileptogenic zone was not adjacent to the eloquent cortex. We conclude from our results that in a considerable number of patients with medically intractable particularly temporal focal epilepsies, resective epilepsy surgery can be based on non-invasive EEG-evaluations and the risk of invasive recordings can be avoided.
Sujet(s)
Cartographie cérébrale , Imagerie diagnostique , Épilepsies partielles/chirurgie , Psychochirurgie , Adolescent , Adulte , Sujet âgé , Enfant , Épilepsies partielles/diagnostic , Épilepsies partielles/physiopathologie , Femelle , Hippocampe/physiopathologie , Hippocampe/chirurgie , Humains , Mâle , Adulte d'âge moyen , Sensibilité et spécificité , Techniques stéréotaxiques , Lobe temporal/physiopathologie , Lobe temporal/chirurgieRÉSUMÉ
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an increasingly recognized autosomal dominant disorder that leads to cerebrovascular manifestations in early adulthood. This study delineates the phenotypic spectrum and the natural history of the disease in 102 affected individuals from 29 families with biopsy-proven CADASIL. Recurrent ischemic episodes (transient ischemic attack [TIA] or stroke) were the most frequent presentation found in 71% of the cases (mean age at onset, 46.1 years; range, 30-66 years; SD, 9.0 years). Forty-eight percent of the cases had developed cognitive deficits. Dementia (28%) was frequently accompanied by gait disturbance (90%), urinary incontinence (86%), and pseudobulbar palsy (52%). Thirty-nine patients (38%) had a history of migraine (mean age at onset, 26.0 years; SD, 8.2 years), which was classified as migraine with aura in 87% of the cases. Psychiatric disturbances were present in 30% of the cases, with adjustment disorder (24%) being the most frequent diagnosis. Ten patients (10%) had a history of epileptic seizures. To delineate the functional consequences of ischemic deficits, we studied the extent of disability in different age groups. The full spectrum of disability was seen in all groups older than age 45. Fifty-five percent of the patients older than age 60 were unable to walk without assistance. However, 14% in this age group exhibited no disability at all. Kaplan-Meier analysis disclosed median survival times of 64 years (males) and 69 years (females). An investigation of the 18 multiplex families revealed marked intrafamilial variations.
Sujet(s)
Artériopathies cérébrales/génétique , Infarctus cérébral/génétique , Accident ischémique transitoire/génétique , Leucoencéphalopathie multifocale progressive/génétique , Adulte , Sujet âgé , Autriche , Artériopathies cérébrales/mortalité , Artériopathies cérébrales/physiopathologie , Infarctus cérébral/mortalité , Infarctus cérébral/physiopathologie , Famille , Femelle , Gènes dominants , Allemagne , Céphalée , Humains , Accident ischémique transitoire/mortalité , Accident ischémique transitoire/physiopathologie , Leucoencéphalopathie multifocale progressive/mortalité , Leucoencéphalopathie multifocale progressive/physiopathologie , Mâle , Adulte d'âge moyen , Migraines , Tests neuropsychologiques , Phénotype , Récidive , Analyse de survie , SyndromeRÉSUMÉ
A 74-year-old woman presented with gait impairment, urinary incontinence, and dementia. She showed lymphocytic CSF pleocytosis and pronounced intrathecal Borrelia burgdorferi antibody production, indicating active Lyme neuroborreliosis. The syndrome of normal-pressure hydrocephalus (NPH) fully remitted after ceftriaxone treatment. Lyme neuroborreliosis may cause NPH by interfering with subarachnoid CSF flow.