Demencia frontotemporal y manía: estudio de un caso y revisión de la literatura

Roberto Sánchez González, Silvia López Zumeta, Miriam Almendrote Muñoz, Luis Prats Sánchez

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INTRODUCTION: Frontotemporal Dementia is a descriptive term used to define a dementia invariably associated with degeneration of the frontal and temporal lobes, in the absence of Alzheimer's disease. It is a heterogeneous group of neurodegenerative diseases with different clinical manifestations, genetic component and anatomopathological alteration. It´s psychiatric symptoms have an insidious onset, with personality deterioration and progressive affectation of behavior. Usually these changes occur before finding abnormalities in neuroimaging studies, their characteristics depend on the location of the cerebral lesions and usually are precede or mask by cognitive disabilities. Apathy and lack of volition are common initial symptoms and occurs in most patients as the disease progresses. Disinhibition is manifested by inappropriate social behaviors such as inadequate laughter, exhibitionism, restless, impulses, uncontrolled sexuality and violence, inappropriate jocularity and childish humor; that symptoms can be present in a half of patients.

CASE REPORT: A representative case report was considered in which the first manifestation of dementia is a syndrome with manic features. Also, specific literature published to date is reviewed.

CONCLUSIONS: Is required a correct differential diagnosis between dementia and the beginning of a late onset bipolar affective disorder, and complementary examinations can be useful for this purpose.

Palabras clave

Demencia frontotemporal; Trastorno bipolar; Trastorno del comportamiento; Trastorno neurológico degenerativo


Neary D, Snowden J, Mann D. Frontotemporal dementia. Lancet Neurol. 2005;4(11):771–80.

Toribio–Díaz ME, Morera–Guitart J. Clinical and biomolecular classification of the frontotemporal dementias. A review of the literature. Rev Neurol. 2008;47:588–98.

Doval O, Gaviria M. Frontotemporal dementia. Actas Esp Psiquiatr. 1999;27:111–26.

Lopez–Pousa S, Garre–Olmo J, Turon–Estrada A, Fajardo–Tibau C, Puig–Puig O, Lozano–Gallego M, Hernandez–Ferrandiz M, Vilalta–Franch J. The clinical incidence of frontal dementia. Rev Neurol. 2002;34(3):216–22.

Weder ND, Aziz R, Wilkins K, Tampi RR. Frontotemporal Dementias: a review. Ann Gen Psychiatry. 2007;6:15.

Iragorri AM. Demencia frontotemporal. Rev Colomb Psiquiatr. 2007;36(1):139–56.

Gálvez–Andres A, Blasco–Fontecilla H, González–Parra S, Molina JD, Padín JM, Rodriguez RH. Secondary bipolar disorder and Diogenes syndrome in frontotemporal dementia: behavioral improvement with quetiapine and sodium valproate. J Clin Psychopharmacol. 2007;27(6):722–3.

Mendez MF, Chen AK, Shapira JS, Lu PH, Miller BL. Acquired extroversion associated with bitemporal variant of frontotemporal dementia. J Neuropsychiatry Clin Neurosci. 2006;18(1):100–7.

Mendez MF, Perryman KM. Neuropsychiatric features of frontotemporal dementia: evaluation of consensus criteria and review. J Neuropsychiatry Clin Neurosci. 2002;14(4):424–9.

Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998;51(6):1546–54.

Zamboni G, Huey ED, Krueger F, Nichelli PF, Grafman J. Apathy and disinhibition in frontotemporal dementia. Insights into their neural correlates. Neurology. 2008;71(10):736–42.

Mendez MF, Lauterbach EC, Sampson SM; ANPA Committee on Research. An evidence–based review of the psychopathology of frontotemporal dementia: a report of the ANPA Committee on Research. J Neuropsychiatry Clin Neurosci. 2008;20(2):130–49.

Rabinovici GD, Miller BL. Frontotemporal Lobar Degeneration. Epidemiology, Pathophysiology, Diagnosis and Management. CNS Drugs. 2010;24(5):375–98.

Hamilton RL. The other dementias: the neuropathology of the non–Alzheimer's disease dementias. Rev Neurol. 2003;37(2):130–9.

Sturm VE, Ascher EA, Miller BL, Levenson RW. Diminished self–conscious emotional responding in frontotemporal lobar degeneration patients. Emotion. 2008 Dec;8(6):861–9.

Allegri RF, Harris P, Feldman M, Taragano F, Paz J. Differential cognitive profiles of fronto–temporal dementia and Alzheimer's disease. Rev Neurol. 1998;27:463–6.

Johns EK, Phillips NA, Belleville S, Goupil D, Babins L, Kelner N, et al. Executive functions in frontotemporal dementia and Lewy body dementia. Neuropsychology. 2009;23(6):765–77.

McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ. Clinical and pathological diagnosis of Frontotemporal Dementia. Arch Neurol. 2001;58:1803–9.

Donoso A, Salinas P. Demencias frontotemporales: tres casos de la variante frontal. Rev Chil Neuro–Psiquiat. 2009; 47 (4): 286–92.

Uterga JM, Rodríguz–Iriarte ML, González–García AI, Taramundi S, Álvaro LC, Cortina C. Dementia with frontal lobe atrophy: clinical study from 18 patients with cognitive impairment and frontal lobe involvement detected by structural and functional neuroimaging. Rev Neurol. 2002;34(8):709–14.

Mendez MF, McMurtray A, Chen AK, Shapira JS, Mishkin F, Miller BL. Functional neuroimaging and presenting psychiatric features in frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2006;77(1):4–7.

Chow TW, Binns MA, Freedman M, Stuss DT, Ramirez J, Scott CJ, Black S. Overlap in frontotemporal atrophy between normal aging and patients with frontotemporal dementias. Alzheimer Dis Assoc Disord. 2008;22(4):327–35.

Seeley WW, Crawford R, Rascovsky K, Kramer JH, Weiner M, Miller BL, Gorno–Tempini ML. Frontal paralimbic network atrophy in very mild behavioral variant frontotemporal dementia. Arch Neurol. 2008;65(2):249–55.

Koedam EL, Van der Flier WM, Barkhof F, Koene T, Scheltens P, Pijnenburg YA. Clinical characteristics of patients with frontotemporal dementia with and without lobar atrophy on MRI. Alzheimer Dis Assoc Disord. 2010;24(3):242–7.

Shulman KI. Disinhibition syndromes, secondary mania and bipolar disorder in old age. J Affect Disord. 1997;46(3):175–82.

Bearden CE, Hoffman KM, Cannon TD. The neuropsychology and neuroanatomy of bipolar affective disorder: a critical review. Bipolar Disord. 2001;3(3):106–50.

Huang SH, Tsai SY, Hsu JL, Huang YL. Volumetric reduction in various cortical regions of elderly patients with early–onset and late–onset mania. Int Psychogeriatr. 2011;23(1):149–54.

Lebert F, Lys H, Haëm E, Pasquier F. Dementia following bipolar disorder. Encephale. 2008;34(6):606–10.

Masouy A, Chopard G, Vandel P, Magnin E, Rumbach L, Sechter D, Haffen E. Bipolar disorder and dementia: where is the link?. Psychogeriatrics. 2011;11(1):60–7.

Arciniegas DB. New–onset bipolar disorder in late life: a case of mistaken identity. Am J Psychiatry. 2006;163(2):198–203.

Gafoor R, O´Keane V. Three case reports of secondary mania: evidence supporting a right frontotemporal locus. Eur Psychiatry. 2003;18(1):32–3.

Woolley JD, Wilson MR, Hung E, Gorno–Tempini ML, Miller BL, Shim J. Frontotemporal dementia and mania. Am J Psychiatry. 2007;164(12):1811–6.

Shimizu H, Hokoishi K, Fukuhara R, Komori K, Ikeda M. Two cases of frontotemporal dementia with predominant temporal lobe atrophy. Psychogeriatrics. 2009;9(4):204–7.

Chan DK, Reutens S, Liu DK, Chan RO. Frontotemporal dementia – features, diagnosis and management. Aust Fam Physician. 2011;40(12):968–72.

Mendez MF. Frontotemporal dementia: therapeutic interventions. Front Neurol Neurosci. 2009;24:168–78.

Kaye ED, Petrovic–Poljak A, Verhoeff NP, Freedman M. Frontotemporal Dementia and Pharmacologic Interventions. J Neuropsychiatry Clin Neurosci. 2010;22(1):19–29.

Portugal Mda G, Marinho V, Laks J. Pharmacological treatment of frontotemporal lobar degeneration: systematic review. Rev Bras Psiquiatr. 2011;33(1):81–90.

Herrmann N, Black SE, Chow T, Cappell J, Tang–Wai DF, Lanctôt KL. Serotonergic Function and Treatment of Behavioral and Psychological Symptoms of Frontotemporal Dementia. Am J Geriatr Psychiatry. 2011 Nov 4. [Epub ahead of print]

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