Evaluation of patients with frontotemporal dementia[FTD] is available at the INR[1-5].
What is Frontotemporal Dementia ?
Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick’s disease, the name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD. Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex. These designations will continue to be debated. As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language. The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic) and includes inappropriate social behavior; lack of social tact; lack of empathy; distractability; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation. The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms. Spatial skills and memory remain intact. There is a strong genetic component to the disease; FTD often runs in families.
TNF is elevated in the cerebrospinal fluid of patients with FTD, and perispinal etanercept may therefore be a potential therapeutic option for selected patients with FTD[1-6]. Clinical improvement in patients with certain forms of frontotemporal dementia following perispinal etanercept at the INR has been reported[1,2,5].
Evaluation and treatment of selected patients with frontotemporal dementia is available at the INR.
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2. Tobinick EL, Gross H. Rapid improvement in verbal fluency and aphasia following perispinal etanercept in Alzheimer’s disease. BMC Neurol, 8, 27 (2008).
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4. Tobinick E. Tumour necrosis factor modulation for treatment of Alzheimer’s disease: rationale and current evidence. CNS Drugs, 23(9), 713-725 (2009).
5. Tobinick E. Perispinal etanercept produces rapid improvement in primary progressive aphasia: identification of a novel, rapidly reversible TNF-mediated pathophysiologic mechanism. Medscape J Med, 10(6), 135 (2008).
6. Sjogren M, Folkesson S, Blennow K, Tarkowski E. Increased intrathecal inflammatory activity in frontotemporal dementia: pathophysiological implications. J Neurol Neurosurg Psychiatry, 75(8), 1107-1111 (2004).