We investigated whether a self-rated anosognosia score can be an indicator for progression of brain atrophy in patients with amyotrophic lateral sclerosis (ALS). Scores for 16 patients were compared with the ventricular areas of the bilateral anterior and inferior horns measured on x-ray computed tomography. Longitudinal enlargement was expressed as a monthly increase in size: (ventricular size at the initial scan – ventricular size at the follow-up scan)/scan interval (months). The anosognosia scores ranged from –4 to 3 and 3–18 in patients with and without frontotemporal lobar degeneration (FTLD), respectively (p = 0.0011). Anosognosia scores were significantly correlated with sizes of anterior (r = 0.704, p = 0.0016) and inferior (r = 0.898, p < 0.0001) horns. In non-demented patients for whom follow-up CT scans were available (n = 7), the scores were significantly correlated with the longitudinal increase in inferior horn size (r = 0.754, p = 0.0496), but not with that of anterior horn size (r = –0.166, p = 0.7111). In conclusion, anosognosia in ALS is associated with greater anterior and inferior horn sizes, reflecting frontotemporal lobar atrophy. Moreover, mild anosognosia in ALS patients without FTLD may predict impending inferior horn enlargement, reflecting medial temporal atrophy.

1.
Mendez MF, Shapira JS: Loss of insight and functional neuroimaging in frontotemporal dementia. J Neuropsychiatry Clin Neurosci 2005;17:413–416.
2.
O’Keeffe FM, Murray B, Coen RF, Dockree PM, Bellgrove MA, Gravan H, Lynch T, Robertson IH: Loss of insight in frontotemporal dementia, corticobasal degeneration and progressive supranuclear palsy. Brain 2007;130:753–764.
3.
Ruby P, Schmit C, Hogge M, D’Argembeau A, Collette F, Salmon E: Social mind representation: where does it fail in frontotemporal dementia? J Cogn Neurosci 2007;19:671–683.
4.
Salmon E, Perani D, Herholz K, Marique P, Kalbe E, Holthoff V, Delbeuk X, Beuthien-Baumann B, Pelati O, Lespagnard S, Collette F, Garraux G: Neural correlates of anosognosia for cognitive impairment in Alzheimer’s disease. Hum Brain Mapping 2006;27:588–597.
5.
Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF: Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998;51:1546–1554.
6.
Woolley SC, Moore DH, Katz JS: Insight in ALS: awareness of behavioral change in patients with and without FTD. Amyotroph Lateral Scler 2010;11:52–56.
7.
Ichikawa H, Koyama S, Ohno H, Ishihara K, Nagumo K, Kawamura M: Writing errors and anosognosia in amyotrophic lateral sclerosis with dementia. Behav Neurol 2008;19:107–116.
8.
Deckel AW, Morrison D: Evidence of a neurologically based ‘denial of illness’ in patients with Huntington’s disease. Arch Clin Neuropsychol 1996;11:295–302.
9.
Ichikawa H, Hieda S, Ohno H, Ohnaka Y, Shimizu Y, Nakajima M, Kawamura M: Kana versus kanji in amyotrophic lateral sclerosis: a clinicoradiological study of writing errors. Eur Neurol 2010;64:148–155.
10.
Ichikawa H, Ohno H, Murakami H, Ohnaka Y, Kawamura M: Writing errors may be a predictive sign for impending brain atrophy progression in amyotrophic lateral sclerosis: a preliminary study using x-ray computed tomography. Eur Neurol 2011;65:346–351.
11.
Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases: El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;1:293–299.
12.
Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A: The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function: BDNF ALS Study Group (Phase III). J Neurol Sci 1999;169:13–21.
13.
Dubois B, Slachevsky A, Litivan I, Pillion B: The FAB: a frontal assessment battery at bedside. Neurology 2000;55:1621–1626.
14.
Rankin KP, Baldwin E, Pace-Savitsky C, Kramer JH, Miller BL: Self-awareness and personality change in dementia. J Neurol Neurosurg Psychiatry 2005;76:632–639.
15.
Zamboni G, Grafman J, Krueger F, Knutson KM, Huey ED: Anosognosia for behavioral disturbances in frontotemporal dementia and corticobasal syndrome: a voxel-based morphometry study. Dement Geriatr Cogn Disord 2010;29:88–96.
16.
Eslinger PJ, Dennis K, Moore P, Antani S, Hauck R, Grossman M: Metacognitive deficits in frontotemporal dementia. J Neurol Neurosurg Psychiatry 2005;76:1630–1635.
17.
Damasio AR, Damasio H, Chui HC: Neglect following damage to frontal lobe or basal ganglia. Neuropsychologia 1980;18:123–132.
18.
Michon A, Deweer B, Pillon B, Agid Y, Dubios B: Relation of anosognosia to frontal lobe dysfunction in Alzheimer’s disease. J Neurol Neurosurg Psychiatry 1994;57:805–809.
19.
Reed BR, Jagust WJ, Coulter L: Anosognosia in Alzheimer’s disease: relationships to depression, cognitive function, and cerebral perfusion. J Clin Exp Neuropsychol 1993;15:231–244.
20.
Starkstein SE, Vazques S, Migliorelli R, Tenson A, Sabe L, Leiguarda R: A single-photon emission computed tomographic study of anosognosia in Alzheimer’s disease. Arch Neurol 1995;52:415–420.
21.
Marshall GA, Kaufer DI, Lopez OL, Rao GR, Hamilton RL, DeKosky ST: Right prosubiculum amyloid plaque density correlates with anosognosia in Alzheimer’s disease. J Neurol Neurosurg Psychiatry 2004;75:1396–1400.
22.
Nakano I, Iwatsubo T, Hashizume Y, Mizutani T, Mannen T: Amyotrophic lateral sclerosis with dementia: lesions in the apical cortex and some deeper structures of the temporal lobe. Neuropathology 1992;12:69–77.
23.
Wightman G, Anderson VER, Martin J, Swash M, Anderton BH, Neary D, Mann D, Luthert O, Leigh PH: Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia. Neurosci Lett 1992;139:269–274.
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