Clinical Neuroscience

[Frontotemporal dementia - Part I History, prevalence, clinical forms]

GALARIOTIS Vasilis, BÓDI Nikoletta, JANKA Zoltán, KÁLMÁN János

MAY 10, 2005

Clinical Neuroscience - 2005;58(05-06)

[The authors report a comprehensive publication consisting of three parts going into the details of history, prevalence, clinical forms, differential diagnosis, genetics, molecular pathomechanism, pathology, clinical diagnosis and treatment of frontotemporal dementia (FTD). The first part of the present review deals with history, prevalence and clinical forms of FTD. The prototypical FTD with circumscribed atrophy was first described by Arnold Pick; Alois Alzheimer found the intraneural inclusions in the patients’ brain. Later it was recognised that many patients had neither the atrophy nor the cellular changes, but genetic mutations have been identified. Frontotemporal dementia is a degenerative condition with unknown etiology in the frontal and anterior temporal lobes of the brain. It is a progressive neurobehavioral syndrome characterized by early decline in social interpersonal conduct, early impairment in the regulation of personal conduct, early emotional blunting, and early loss of insight. There are no reliable epidemiological studies on the prevalence of FTD, but it is well-accepted that FTD is a common cause for dementia before the age of 65 (it constitues approximately five percent of all irreversible dementias). The nomenclature of the FTD has been confusing and continues to be. Three major clinical syndromes can be identified: 1 frontal variant FTD (dementia of frontal type) in which changes in social behavior and personality predominate, 2. in semantic dementia (progressive fluent aphasia) there is a breakdown in the conceptual database which underlies language production and comprehension, 3. in progressive nonfluent aphasia the phonologic and syntactic components of language are affected. The authors report two cases, which can point to clinical symptoms and forms, and mention the problems of the differential diagnosis and therapy.]

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[Pathophysiology of restless leg syndrome and periodic leg movement disorder in view of the latest research findings]

KÖVES Péter, SZAKÁCS ZOLTÁN

[Both restless leg syndrome and periodic leg movement disorder have been classified as primary sleep disorders by the International Classification of Sleep Disorders. Considering the characteristic clinical symptoms, it is supposed that their pathomechanism involves the peripheral and central stimulusprocessing mechanisms of the nervous system as well as several elements of the motor system. During the last couple of years many new elements of the pathomechanism have been discovered, in particular the dysfunction of the postsynaptic dopamin receptors related to the iron metabolism of the central nervous system, the role of opiate receptors, and the involvement of subclinical small fiber neuropathy. Many of these findings have been incorporated into the diagnostic and treatment protocols used in the management of patients with restless leg syndrome or periodic leg movement disorder. Considering the rapidly increasing number of publications on their pathomechanism and the various fields it involves, the authors found it necessary to evaluate these data and to interpret their relationships within the frame of sleep-wake regulation.]

Clinical Neuroscience

[THE SELECTIVE COX-2 INHIBITOR MELOXICAM AND SALICYLATE THERAPY]

TÓTH Károly

[Although during treatment of arthrosis with meloxicam the level of thromboxan A2 decreases, thrombocyte functions are not affected. Meloxicam in therapeutic doses doesn’t increase the risk of haemorrhage. Previously it was suspected that coadministration of salicylates with certain other non-steroid antiinflammatory drugs (NSAIDs) will suppress the effect of salicylate. Van Ryn et al have proved that this is not the case with salicylate plus meloxicam therapy. It is hypothesized that meloxicam loosely binds to the cyclooxygenase-1 (COX-1) enzyme and salicylate can easily replace it.]

Clinical Neuroscience

[Primary prevention program of the Hungarian Spine Society - Part II Controlled prospective study of short term efficacy]

SOMHEGYI Annamária, TÓTH János, MAKSZIN Imre, GARDI Zsuzsa, FESZTHAMMER Artúrné, DARABOSNÉ Tim Irma, TÓTHNÉ Steinhausz Viktória, TÓTHNÉ Szabó Klára, VARGA Péter Pál

[Introduction - The primary prevention program of the Hungarian Spine Society is set around 12 test exercises that assess performance of postural muscles and then develops their strength and flexibility. By this the scheme aims to make the biomechanically correct use of the spinal column spontaneous. Subjects and methos - The application of the scheme in the frame of physical education in a primary school was investigated in a prospective controlled study in the school-year of 2001/2002. Participating children were aged 6 to 14. The intervention group (n=200) regularly did the posture correction exercises with their teacher's direction in physical education classes, while the control group (n=213) did not. The 12 test exercises of the posture correction scheme of the Hungarian Spine Society were tested by an independent physiotherapist at the beginning and at the end of the school-year in both groups. Results were analyzed by paired and unpaired t-tests and by a chi-square test. Results - At the end of the school-year the strength and flexibility of the postural muscles of the intervention group improved significantly compared both to their own performance (p<0.01) at the beginning of the school-year and to the control group. Test results of the control group were significantly (p<0.05) worse at the end of the school-year than their own results at beginning of the school-year, and were more significantly (p<0.01) worse than the test results of intervention group. Conclusion - The results of the controlled study confirm that regular use of the preventive exercises in physical education improves the strength and flexibility of postural muscles. In order to analyze the results concerning specific muscle groups or age groups a detailed study with age- and gender-matched controls will be necessary.]

Clinical Neuroscience

[A case history and diagnostical rewiev of primary cerebral angiitis]

CSÉPÁNY Tünde, KOLLÁR József, SIKULA Judit, MOLNÁR Mária, CSIBA László

[The authors present a case history of primary cerebral angiitis with four years of follow-up. The early diagnosis was based on typical clinical symptoms, brain MRI, intracerebral MRA and histology of sural nerve biopsy. Electroneurography suggested peripheral involvement, although the patient did not have clinical signs of peripheral neuropathy. Glucocorticoid and immunosuppressive treatment resulted in remission. The diagnostic difficulties of primary cerebral vasculitis are also summarized in the discussion.]

Clinical Neuroscience

[Testing the administration of cannabinoids in neurological diseases]

All articles in the issue

Related contents

Clinical Neuroscience

[Frontotemporal dementia or frontotemporal lobar degeneration - Overview of a group of proteinopathies]

CATHERINE Haberland

[Frontotemporal dementia is the second most common early onset dementia after Alzheimer disease. Frontotemporal dementias are a complex group of dementias. The clinical diagnosis can be perplexing because of concurring psychiatric and neurologic syndromes. Frontotemporal lobar degeneration, the underlying pathology, represents an emerging group of proteinopathies. Genetic factors play an important part in the etiologies of dementias. This article overviews current defining characteristics of frontotemporal dementias known also as frontotemporal lobar degenerations]

Clinical Neuroscience

[Two cases of frontotemporal dementia]

SZABÓ Erzsébet, SZABÓ Mihály

[Frontotemporal dementias represent the third most common cause of primer degenerative dementias next to Alzheimer’s disease and Lewy body disease. Frontotemporal dementia constitutes 10-20% of all praesenilis dementias. The authors present the results of the 10 years' clinical, neuropsychological, neuropathological examinations and brain imaging with the examples of two cases. At the early stage of frontotemporal dementia changes of personality and social conduct are prominent, whereas cognitive functions are relativelly well preserved. The usual dementia tests are not sufficiently sensitive to disclose noncognitive symptoms. Clinical diagnosis as well as differentiation from functional psychiatric disorders can be difficult. Brain imaging present the frontal and the anterior temporal lobe atrophy and selective hypometabolism in these areas. The typical onset is between at the age 50 and 65 years. It is very rare under the age of 30. The symptoms of two patients started at the age of 42-44. The first diagnosis was post traumatic stress disorder. Later stereotyped behaviour, mental rigidity, hyperorality, irritability, progressive reduction of speech and vegetative dysfunctions appeared. Besides the affecting of the irresistibly worsening symptoms and the medical care requiring strength and inventiveness, the authentic informing of the relatives is also a challenge. The caregivers have special relationship with the patients and their relatives.]

Clinical Neuroscience

[Frontotemporal dementia, Pick’s disease]

ANDREW Kertesz

[A significant expansion of knowledge in the last few years, especially in the molecular biology of frontotemporal dementia (FTD) is summarized. This condition, formerly known as Pick’s disease and considered rare, is estimated to be 12-15% of all dementias and 30-50% early onset ones. The clinical picture is protean, mainly a behavioural and language impairment, but the extrapyramidal syndromes of CBD and PSP also belong. These seemingly different presentations converge, as one or other areas in the brain are affected. Less than half of the cases are tauopathies, the majority has been discovered to have a TDP-43 and most recently a FUS proteinopathy, shared with ALS, opening potential opportunities for pharmacological approaches to treatment. Tau and progranulin mutations on Ch-17 and some others, point to molecular mechanisms. A glossary is provided to navigate the complex terminology.]