Table 5

Main neuropsychological profiles in GRN mutation carriers

PatientsDisease duration at examinationMain disorders (diagnosis)Episodic memory disordersSemantic disordersAphasiaSigns of parietal dysfunction
Patients with isolated frontal behavioural and cognitive syndrome (mean disease duration 2.3 years)
F057/0071Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F521/0102Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F587/0065Behavioural (fvFTD)Frontal type
F741/0072Behavioural (fvFTD)Frontal typePossible(Reduction of spontaneous language)na
F716/0012Behavioural (fvFTD)Frontal typePresent(Reduction of spontaneous language)
F329/0012Behavioural (fvFTD)Frontal type
Patients with frontal syndrome and parietal signs (mean disease duration: 2.8 years)
F043/0013Behavioural (fvFTD)Frontal typenaReduced fluency, semantic paraphasias, verbal comprehension deficitConstructional disorders ideomotor, ideational and dressing apraxia
F263/0013Behavioural and language (fvFTD)naPNFA with agrammatism and speech apraxiaConstructional disorders
F128/0013Behavioural (fvFTD)Frontal typeConstructional disorders
F171/0013Behavioural (fvFTD)(Reduction of spontaneous language)Ideomotor and dressing apraxia, Constructional disorders, L spatial, sensory and motor hemineglect, visual agnosia
F171/0022Behavioural and language (fvFTD)Frontal typePNFA with agrammatismApraxic agraphia, constructional disorders, dyscalculia
F129/0012Behavioural (fvFTD)na(Reduction of spontaneous language)Ideomotor and dressing apraxia
F540/0023CBSFrontal type(Reduction of spontaneous language)ideomotor apraxia and constructional disorders L spatial, sensory, motor hemineglect
F540/0013CBSFrontal type(Reduction of spontaneous language)Constructional disorders, Left spatial and motor hemineglect, visual agnosia
Patients with amnestic hippocampal syndrome (mean disease duration 2.5 years)
F015/0102Memory disorders (AD)Hippocampal typeMild word finding difficulties
F129/0021Memory disorders (AD)Hippocampal typeConstructional disorders
F160/0192Memory disorders (AD)Hippocampal type
F524/0012Psychiatric presentation (fvFTD)Hippocampal typeProsopagnosia Semantic verbal deficit
Patients with progressive aphasia at onset (mean disease duration: 2.5 years)
F171/0043Language (PPA)naConduction aphasia (phonemic paraphasias, impaired repetition, preserved comprehension)Ideomotor apraxia and constructional disorders Spatial working memory impaired
F716/0023Language (PPA)nanaFluent aphasia with comprehension (verbal semantic) deficit
F023/0042Language (PPA)Reduced fluency with semantic paraphasias, without agrammatism
F258/0012Language (PPA)Hippocampal typeFluent aphasia with comprehension (verbal semantic) deficitConstructional disorders
PatientsDisease duration at examinationMain disorders (diagnosis)Episodic memory disordersSemantic disordersAphasiaSigns of parietal dysfunction
Patients with isolated frontal behavioural and cognitive syndrome (mean disease duration 2.3 years)
F057/0071Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F521/0102Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F587/0065Behavioural (fvFTD)Frontal type
F741/0072Behavioural (fvFTD)Frontal typePossible(Reduction of spontaneous language)na
F716/0012Behavioural (fvFTD)Frontal typePresent(Reduction of spontaneous language)
F329/0012Behavioural (fvFTD)Frontal type
Patients with frontal syndrome and parietal signs (mean disease duration: 2.8 years)
F043/0013Behavioural (fvFTD)Frontal typenaReduced fluency, semantic paraphasias, verbal comprehension deficitConstructional disorders ideomotor, ideational and dressing apraxia
F263/0013Behavioural and language (fvFTD)naPNFA with agrammatism and speech apraxiaConstructional disorders
F128/0013Behavioural (fvFTD)Frontal typeConstructional disorders
F171/0013Behavioural (fvFTD)(Reduction of spontaneous language)Ideomotor and dressing apraxia, Constructional disorders, L spatial, sensory and motor hemineglect, visual agnosia
F171/0022Behavioural and language (fvFTD)Frontal typePNFA with agrammatismApraxic agraphia, constructional disorders, dyscalculia
F129/0012Behavioural (fvFTD)na(Reduction of spontaneous language)Ideomotor and dressing apraxia
F540/0023CBSFrontal type(Reduction of spontaneous language)ideomotor apraxia and constructional disorders L spatial, sensory, motor hemineglect
F540/0013CBSFrontal type(Reduction of spontaneous language)Constructional disorders, Left spatial and motor hemineglect, visual agnosia
Patients with amnestic hippocampal syndrome (mean disease duration 2.5 years)
F015/0102Memory disorders (AD)Hippocampal typeMild word finding difficulties
F129/0021Memory disorders (AD)Hippocampal typeConstructional disorders
F160/0192Memory disorders (AD)Hippocampal type
F524/0012Psychiatric presentation (fvFTD)Hippocampal typeProsopagnosia Semantic verbal deficit
Patients with progressive aphasia at onset (mean disease duration: 2.5 years)
F171/0043Language (PPA)naConduction aphasia (phonemic paraphasias, impaired repetition, preserved comprehension)Ideomotor apraxia and constructional disorders Spatial working memory impaired
F716/0023Language (PPA)nanaFluent aphasia with comprehension (verbal semantic) deficit
F023/0042Language (PPA)Reduced fluency with semantic paraphasias, without agrammatism
F258/0012Language (PPA)Hippocampal typeFluent aphasia with comprehension (verbal semantic) deficitConstructional disorders

na = not available; AD = Alzheimers disease; CBDS = corticobasal syndrome; fvFTD = frontal variant of FTD; L = left; LBD = Lewy body dementia; PNFA = progressive non-fluent aphasia; PPA = primary progressive aphasia; UL = upper limb; − = absent.

Table 5

Main neuropsychological profiles in GRN mutation carriers

PatientsDisease duration at examinationMain disorders (diagnosis)Episodic memory disordersSemantic disordersAphasiaSigns of parietal dysfunction
Patients with isolated frontal behavioural and cognitive syndrome (mean disease duration 2.3 years)
F057/0071Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F521/0102Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F587/0065Behavioural (fvFTD)Frontal type
F741/0072Behavioural (fvFTD)Frontal typePossible(Reduction of spontaneous language)na
F716/0012Behavioural (fvFTD)Frontal typePresent(Reduction of spontaneous language)
F329/0012Behavioural (fvFTD)Frontal type
Patients with frontal syndrome and parietal signs (mean disease duration: 2.8 years)
F043/0013Behavioural (fvFTD)Frontal typenaReduced fluency, semantic paraphasias, verbal comprehension deficitConstructional disorders ideomotor, ideational and dressing apraxia
F263/0013Behavioural and language (fvFTD)naPNFA with agrammatism and speech apraxiaConstructional disorders
F128/0013Behavioural (fvFTD)Frontal typeConstructional disorders
F171/0013Behavioural (fvFTD)(Reduction of spontaneous language)Ideomotor and dressing apraxia, Constructional disorders, L spatial, sensory and motor hemineglect, visual agnosia
F171/0022Behavioural and language (fvFTD)Frontal typePNFA with agrammatismApraxic agraphia, constructional disorders, dyscalculia
F129/0012Behavioural (fvFTD)na(Reduction of spontaneous language)Ideomotor and dressing apraxia
F540/0023CBSFrontal type(Reduction of spontaneous language)ideomotor apraxia and constructional disorders L spatial, sensory, motor hemineglect
F540/0013CBSFrontal type(Reduction of spontaneous language)Constructional disorders, Left spatial and motor hemineglect, visual agnosia
Patients with amnestic hippocampal syndrome (mean disease duration 2.5 years)
F015/0102Memory disorders (AD)Hippocampal typeMild word finding difficulties
F129/0021Memory disorders (AD)Hippocampal typeConstructional disorders
F160/0192Memory disorders (AD)Hippocampal type
F524/0012Psychiatric presentation (fvFTD)Hippocampal typeProsopagnosia Semantic verbal deficit
Patients with progressive aphasia at onset (mean disease duration: 2.5 years)
F171/0043Language (PPA)naConduction aphasia (phonemic paraphasias, impaired repetition, preserved comprehension)Ideomotor apraxia and constructional disorders Spatial working memory impaired
F716/0023Language (PPA)nanaFluent aphasia with comprehension (verbal semantic) deficit
F023/0042Language (PPA)Reduced fluency with semantic paraphasias, without agrammatism
F258/0012Language (PPA)Hippocampal typeFluent aphasia with comprehension (verbal semantic) deficitConstructional disorders
PatientsDisease duration at examinationMain disorders (diagnosis)Episodic memory disordersSemantic disordersAphasiaSigns of parietal dysfunction
Patients with isolated frontal behavioural and cognitive syndrome (mean disease duration 2.3 years)
F057/0071Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F521/0102Behavioural (fvFTD)Frontal type(Reduction of spontaneous language)
F587/0065Behavioural (fvFTD)Frontal type
F741/0072Behavioural (fvFTD)Frontal typePossible(Reduction of spontaneous language)na
F716/0012Behavioural (fvFTD)Frontal typePresent(Reduction of spontaneous language)
F329/0012Behavioural (fvFTD)Frontal type
Patients with frontal syndrome and parietal signs (mean disease duration: 2.8 years)
F043/0013Behavioural (fvFTD)Frontal typenaReduced fluency, semantic paraphasias, verbal comprehension deficitConstructional disorders ideomotor, ideational and dressing apraxia
F263/0013Behavioural and language (fvFTD)naPNFA with agrammatism and speech apraxiaConstructional disorders
F128/0013Behavioural (fvFTD)Frontal typeConstructional disorders
F171/0013Behavioural (fvFTD)(Reduction of spontaneous language)Ideomotor and dressing apraxia, Constructional disorders, L spatial, sensory and motor hemineglect, visual agnosia
F171/0022Behavioural and language (fvFTD)Frontal typePNFA with agrammatismApraxic agraphia, constructional disorders, dyscalculia
F129/0012Behavioural (fvFTD)na(Reduction of spontaneous language)Ideomotor and dressing apraxia
F540/0023CBSFrontal type(Reduction of spontaneous language)ideomotor apraxia and constructional disorders L spatial, sensory, motor hemineglect
F540/0013CBSFrontal type(Reduction of spontaneous language)Constructional disorders, Left spatial and motor hemineglect, visual agnosia
Patients with amnestic hippocampal syndrome (mean disease duration 2.5 years)
F015/0102Memory disorders (AD)Hippocampal typeMild word finding difficulties
F129/0021Memory disorders (AD)Hippocampal typeConstructional disorders
F160/0192Memory disorders (AD)Hippocampal type
F524/0012Psychiatric presentation (fvFTD)Hippocampal typeProsopagnosia Semantic verbal deficit
Patients with progressive aphasia at onset (mean disease duration: 2.5 years)
F171/0043Language (PPA)naConduction aphasia (phonemic paraphasias, impaired repetition, preserved comprehension)Ideomotor apraxia and constructional disorders Spatial working memory impaired
F716/0023Language (PPA)nanaFluent aphasia with comprehension (verbal semantic) deficit
F023/0042Language (PPA)Reduced fluency with semantic paraphasias, without agrammatism
F258/0012Language (PPA)Hippocampal typeFluent aphasia with comprehension (verbal semantic) deficitConstructional disorders

na = not available; AD = Alzheimers disease; CBDS = corticobasal syndrome; fvFTD = frontal variant of FTD; L = left; LBD = Lewy body dementia; PNFA = progressive non-fluent aphasia; PPA = primary progressive aphasia; UL = upper limb; − = absent.

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