Cognitive Impairment Involving Social Cognition in SPG4 Hereditary Spastic Paraplegia.
Objectives. To describe cognitive assessment including social cognition in SPG4 patients.Methods. We reported a series of nine patients with SPG4 mutation with an extensive neuropsychological examination including social cognition assessment.Results. None of our patients presented with mental retardation or dementia. All presented with mild cognitive impairment with a high frequency of attention deficit (100%), executive disorders (89%), and social cognition impairment (78%). An asymptomatic patient for motor skills presented with the same cognitive profile. No correlation was found in this small sample between cognitive impairment and motor impairment, age at disease onset, or disease duration.Conclusions. SPG4 phenotypes share some cognitive features of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Cognitive disorders including executive disorders and social cognition impairment are frequent in SPG4 patients and might sometimes occur before motor disorders. Therefore, cognitive functions including social cognition should be systematically assessed in order to improve the clinical management of this population.
University Hospital of Besançon, CH Jean Minjoz, Besançon, France.
Ludivine
L
Chamard
University Hospital of Besançon, CH Jean Minjoz, Besançon, France.
Sabrina
S
Ferreira
Regional Hospital of Dole, CH Louis Pasteur, Dole, France.
Alexa
A
Pijoff
University Hospital of Besançon, CH Jean Minjoz, Besançon, France.
Manon
M
Silvestre
University Hospital of Besançon, CH Jean Minjoz, Besançon, France.
Eric
E
Berger
University Hospital of Besançon, CH Jean Minjoz, Besançon, France.
Eloi
0000-0003-3036-9266
E
Magnin
Neurology. 2000 Jul 12;55(1):89-94
10891911
Brain. 1998 Apr;121 ( Pt 4):601-9
9577388
Neuropsychology. 2012 Jan;26(1):81-90
21895376
Clin Neurol Neurosurg. 1992;94 Suppl:S16-8
1320499
Neurology. 2004 Feb 10;62(3):407-10
14872021
Neurology. 2001 May 8;56(9):1133-42
11342677
Eur J Hum Genet. 2008 Jan;16(1):97-104
17957230
PLoS One. 2011;6(10):e25948
21998727
Rev Neurol (Paris). 2010 Jun-Jul;166(6-7):606-14
20117809
Arch Neurol. 2003 Aug;60(8):1113-8
12925368
J Intern Med. 2004 Sep;256(3):183-94
15324362
J Neurol Sci. 2014 Jan 15;336(1-2):191-6
24239183
J Neurol Sci. 2009 Feb 15;277(1-2):124-9
19084842
Gerontologist. 1969 Autumn;9(3):179-86
5349366
Neuropsychology. 2011 Jan;25(1):53-65
20919762
Brain. 1996 Oct;119 ( Pt 5):1487-96
8931574
Neurology. 2009 Aug 4;73(5):378-84
19652142
J Neurol Neurosurg Psychiatry. 2012 Apr;83(4):411-6
22291219
Neurology. 2000 Apr 11;54(7):1510-7
10751268
J Neurol Sci. 2005 Aug 15;235(1-2):23-32
15939438