Adam E. Klein, MD - West Virginia State Medical Association

| Scientific Articlepharmacotherapy and supportiveservices were established.DiscussionFrontotemporal lobar dementia is aneuropathological disorder involvingdegeneration of the frontal and/ortemporal lobes, sometimes involvingthe parietal lobes. Age of onset ismost common between 45 and 65.There is an equal incidence betweenmen and women. 2 The most commonclinical findings include personalitychanges, dramatic changes insocial conduct and behaviors,and language abnormalities.Frontotemporal lobar degenerationis a diagnostic term that encompassesmultiple distinct forms of thisdementia syndrome, with the threemajor subtypes being frontotemporaldementia, semantic dementia,and primary progressive aphasia.For purposes of this case report,the frontotemporal dementiabehavioral variant will be the focus.Frontotemporal dementia behavioralvariant is the most commondiagnostic subgroup, with overhalf of the diagnoses falling underthis category. 3 It has been furtherproposed that among the behavioralvariants of frontotemporal dementia,distinct anatomical patterns of greymatter loss directly correlate withan even more detailed classificationof the behavioral variant subtype.Studies have demonstrated thatdepending on the general locationof atrophy, several variants ofbehavior abnormalities can result.For example, frontal dominateatrophy is found to have significantdysfunction of executive functions.Temporal dominate atrophy hasclinical findings with significantlyworse memory performance. Wheninvolved, parietal lobe atrophy oftenpresents with aphasia. 1 It is criticalto keep these behavioral variantsubtypes in mind when evaluatingthe above patient. When the patientinitially presented, there were onlysubtle findings of fronto-temporalparietalatrophy on MRI imaging.However, with disease advancementevident by clinical deterioration,repeat imaging revealed significantprogression in atrophy of the frontaland temporal lobes, though withgeneralized atrophy being noted.In hindsight, the findings of thispatient’s executive dysfunctiondirectly correlated with hisfrontotemporal atrophy. However,arriving at this patient’s diagnosiswas a difficult task for variousreasons. First of all, his youth atage 44 was atypical in itself forfrontotemporal lobar dementia.His concurrent use of alcohol alsoconfounded the evaluators, given thatintoxication and recurrent use clearlyimpact executive functions andmemory. Additionally, the symptomsof grandiosity, distractibility,psychomotor agitation, and histhoughtless behaviors with gratifyinga need to engage in pleasurableactivity, all lead evaluations towardan alternative diagnosis of a manicepisode, and consequently adiagnosis of bipolar disorder. It wasnot until after disease progression inspite of multiple medication trials,and repeated imaging demonstratingthe increased brain atrophy, didthe diagnosis of frontotemporallobar degeneration declare itself.ConclusionFrontotemporal lobar dementiais a relatively rare disorder thatpresents with a number of symptoms,many of which overlap thosesimilar to mania, psychosis, andsubstance abuse disorders. Whenclinicians do not constantly maintaina differential diagnosis, especiallyin patients resistant to appropriatepharmacotherapy, the diagnosis canoften be delayed or even missed.References1. Whitwell JL, Przybelski SA, Weigand SD,Ivnik RJ, Vermuri P, Gunter JL, Senjem ML,Shiung MM, BoeveBF, Knopman DS,ParisiJE, Dickson DW, Peterson RC, Jack CR,Josephs KA: Distinct anatomical subtypesof the behavioural variant of frontotemporaldementia: a cluster analysis study. Brain: AJournal of Neurology 2009; 232-247.2. Snowden JS, Neary D, Mann DMA:Frontotemporal Dementia. British Journal ofPsychiatry 2002; 140-143.3. Bozoki AC, Farooq MU: Frontotemporallobar degeneration: Insights fromneuropsychology and neuroimaging.International Review of Neurobiology, Vol84; 185-213.4. Kertesz A, Munoz D: Relationship betweenfrontotemporal dementia and corticobasilarand corticobasilar degeneration/Progressivesupranuclear palsy. Dementia and GeriatricCognitive Disorders 2004; 17:282-286.5. Joseph R: Frontal lobe psychopathology:mania, depression, confabulation,catatonia, perseveration, obsessivecompulsions, and schizophrenia.Psychiatry, 1999; 62: 138-72.6. Giordana MT, Ferrero P, Grifoni S, PellerinoA, Naldi A, Montuschi A: Dementia andcognitive impairment in amyotrophic lateralsclerosis: a review. Neurology Science, Nov2009.Fast &Simple!On-line DuesPayment NowAvailable!wvsma.org(scan code to go directly to online payment center)July/August 2012 | Vol. 108 17