BackgroundFrontotemporal dementia (FTD) is one of the most frequent dementia types in patients under 65 years of age. Currently, no therapy can effectively improve the cognitive deficits associated with FTD. Repetitive transcranial magnetic stimulation (rTMS) is a noninvasive method of inducing brain plasticity with therapeutic potential in neurodegenerative diseases. The purpose of this study was to evaluate the effect of rTMS on cognitive, behavioral, and emotional function in FTD.MethodsNine patients (seven women, four men, mean age 61.7±10.1 years) with the behavioral variant of FTD, one with nonfluent/agrammatic variant primary progressive aphasia, and one with progressive nonfluent aphasia (subtypes of FTD) underwent 10 daily sessions of 10 Hz rTMS over the bilateral dorsolateral prefrontal cortex. Cognitive and behavioral assessments were administered before and after therapy.ResultsAfter rTMS, the Montreal Cognitive Assessment and letter and digit cancellation test scores, as well as reading time and error number in the Stroop test improved. The caregivers’ impression of the daily functioning of patients improved in the Frontal Behavioral Inventory scores. These changes were not paralleled by an improvement of mood.ConclusionThe results indicate that rTMS may improve the cognitive performance of patients with FTD and warrant sham-controlled trials.
Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy with the fatal outcome, caused by the accumulation of pathological prion protein in the central nervous system (CNS). CJD is classified into four types: sporadic (sCJD), familial or genetic (fCJD), iatrogenic (iCJD) and variant form (vCJD). The recognition of CJD is based on the clinical presentation, neuroimaging, electroencephalography and biochemical tests. The hyperintense signals in basal ganglia on brain magnetic resonance imaging (MRI), periodic sharp and slow wave complexes (PSWCs) in the electroencephalogram as well as presence of neuronal proteins such as protein 14-3-3 in the cerebrospinal fluid (CSF) support the diagnosis. The definite diagnosis of CJD still demands neuropathological confirmation.We report the case of a 56-year-old woman with the rapidly progressive cognitive impairment, motor dysfunctions and the fulminant neurological deterioration to akinetic mutism during the five weeks' hospitalisation. The probable diagnosis of sCJD was based on medical history and characteristic findings in MRI. The positive result of the real-time quaking-induced conversion (RT-QuIC) test and presence of protein 14-3-3 were obtained post-mortem and definite diagnosis was confirmed by neuropathological examination. In this paper we would like to emphasize the difficulties in reaching the diagnosis and the need for a series of diagnostic examinations in different points of time to obtain the confirming results.
Nowotworowe zajęcie opon mózgowo-rdzeniowych jest rzadko występującym powikłaniem choroby nowotworowej o niekorzystnym rokowaniu. Największe ryzyko zajęcia opon mózgowo-rdzeniowych odnotowano w przypadku nowotworów układu krwiotwórczego, raka sutka, raka płuca, czerniaka i gruczolakoraków przewodu pokarmowego. Zróżnicowana manifestacja kliniczna nowotworowego zapalenia opon mózgowo-rdzeniowych wynika z równoczesnego i wielopoziomowego zajęcia układu nerwowego, co implikuje wdrożenie szerokiej diagnostyki różnicowej. Rozpoznanie ustala się, opierając się na korelacji obrazu klinicznego z wynikami badań dodatkowych, w szczególności badania analitycznego i cytologicznego płynu mózgowo-rdzeniowego oraz badania rezonansu magnetycznego z kontrastem. W niniejszej publikacji zaprezentowano przypadek 59-letniej pacjentki z nowotworowym zapaleniem opon mózgowo-rdzeniowych, które stanowiło pierwszą manifestację gruczolakoraka płuca.Słowa kluczowe: nowotworowe zapalenie opon mózgowo-rdzeniowych, meningoza, karcynomatoza, rakowatość opon, gruczolakorak płuca Carcinomatous meningitis is a rare complication of a malignant disease with a fatal outcome. The greatest predilection for neoplastic meningitis was observed in the course of hematological malignancies, breast cancer, lung cancer, melanoma and gastrointestinal adenocarcinomas. The simultaneous and multilevel nervous system involvement results in the diverse clinical manifestation, therefore it requires a wide differential diagnosis. The diagnosis is based on the correlation of the clinical presentation with the results of additional tests, especially the examination of cerebrospinal fluid and magnetic resonance imaging with contrast. We present the case of 59-year old woman with the carcinomatous meningitis as the first manifestation of lung adenocarcinoma.
Purpose: The initial presentation of autoimmune encephalitis (AIE) may resemble other disorders and a comprehensive investigation is required. In this paper we emphasize the challenges faced by a treating team performing a differential diagnosis and the necessity for insightful multidisciplinary collaboration. Case description: We report on the case of a 67-year old man who developed transient disturbances of consciousness in the course of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, which was the first manifestation of malignant myeloma. To our knowledge, this is the first case of anti-NMDAR encephalitis associated with multiple myeloma described in the literature. Comment: AIE with anti-NMDAR antibodies has become a well-characterised variant of the condition. AIE may or may not be associated with tumour; therefore it is necessary to use the screening approach for primary malignancy. A proper diagnosis is essential in management due to AIE's potential to respond to immunotherapy and tumour removal if one is present.
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