Dementia in motor neuron disease: reviewing the role of MRI in diagnosis

The superimposed clinical features of motor neuron disease (MND) and frontotemporal dementia (FTD) comprise a distinct, yet not fully understood, neurological overlap syndrome whose clinicopathological basis has recently been reviewed. Here, we present a review of the clinical, pathological and genetic basis of MND-FTD and the role of MRI in its diagnosis. In doing so, we discuss current techniques that depict the involvement of the selective corticospinal tract (CST) and temporal lobe in MND-FTD.

Key words:
frontotemporal dementia; magnetic resonance; motor neuron disease; amyotrophic lateral sclerosis; frontotemporal lobe degeneration

Authorship

Antonio José da Rocha

PhD, Division of Neuroradiology - Santa Casa de Misericórdia de São Paulo, Brazil.Santa Casa de Misericórdia de São PauloBrazilBrazilPhD, Division of Neuroradiology - Santa Casa de Misericórdia de São Paulo, Brazil.

MD, Division of Neuroradiology - Fleury Medicina e Saúde (São Paulo - Brazil)Fleury Medicina e SaúdeBrazilSão Paulo, BrazilMD, Division of Neuroradiology - Fleury Medicina e Saúde (São Paulo - Brazil)

Renato Hoffmann Nunes

Antonio Carlos Martins Maia Jr.

.Antonio José da Rocha. Santa Casa de Misericordia de São Paulo / Serviço de Diagnostico por Imagem - Rua Dr. Cesário Motta Junior, 112 - 01221-020 São Paulo SP - Brazil. E-mail: a.rocha@uol.com.br

Author contributions. Dr. Rocha was responsible for the study concept, critical revision of the manuscript and for imaging selection. Dr. Maia Jr and Dr. Nunes were responsible for the literature review and writing the manuscript

Disclosure: The authors report no conflits of interest

SCIMAGO INSTITUTIONS RANKINGS

Figures | Tables

Figures (2)
Tables (2)

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Figure 1
A man of 42 years of age presented with UMN + LMN signs associated with dementia and language-speech abnormalities (his mother had died at 67 years old with a diagnosis of ALS-FTD). [A] Axial T1 MTC image showed an abnormally selective hyperintensity throughout the corticospinal tracts, predominantly on the left side, assuming a typical 'W-like appearance' crossing the corpus callosum (arrowheads). [B] Coronal FLAIR image depicted a marked atrophy in the left temporal pole with blurring of the grey/white matter differentiation (arrows). Note the abnormal hyperintensity in the left amygdala and hippocampus.

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Figure 2
A man of 61 years of age presented with a history of progressive non-fluent aphasia in the four months prior to admission. This was followed by an asymmetrical flaccid tetraparesis, hyperreflexia in all four limbs, which was associated with a bilateral Hoffmann sign, and fasciculation. [A] Axial T1 MTC image showed an abnormal hyperintensity involving the cortical and subcortical frontal areas including pre-central gyri and the remaining subcortical regions of the frontal lobes. [B-C] Axial and coronal FLAIR images depicted left temporal lobe atrophy with blurring of the grey/white matter differentiation (arrows). [D] Brain macroscopy confirmed atrophy in the left temporal lobe (arrow). Left hippocampus cortex showed neuronal loss as well as cytoplasmic, nuclear and extracellular UI inclusions, in addition to neuronal loss and astrogliosis (not shown).

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Table 1
A summary of genetic, clinical and brain histopathology data together with the possible target of mutations in ALS and/or FTD.²⁰20. HARDY J, ROGAEVA E. MOTOR NEURON DISEASE AND FRONTOTEMPORAL DEMENTIA: SOMETIMES RELATED, SOMETIMES NOT. EXP NEUROL 2014;262 PT B: 75-83. ⁸²82. NG ASL, RADEMAKERS R, MILLER BL. FRONTOTEMPORAL DEMENTIA: A BRIDGE BETWEEN DEMENTIA AND NEUROMUSCULAR DISEASE. ANN NY ACAD SCI 2014; 1338:71-93.

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Table 2
Defining cognitive and behavioural subtypes in ALS.⁸³

Figure 1 A man of 42 years of age presented with UMN + LMN signs associated with dementia and language-speech abnormalities (his mother had died at 67 years old with a diagnosis of ALS-FTD). [A] Axial T1 MTC image showed an abnormally selective hyperintensity throughout the corticospinal tracts, predominantly on the left side, assuming a typical 'W-like appearance' crossing the corpus callosum (arrowheads). [B] Coronal FLAIR image depicted a marked atrophy in the left temporal pole with blurring of the grey/white matter differentiation (arrows). Note the abnormal hyperintensity in the left amygdala and hippocampus.

Figure 2 A man of 61 years of age presented with a history of progressive non-fluent aphasia in the four months prior to admission. This was followed by an asymmetrical flaccid tetraparesis, hyperreflexia in all four limbs, which was associated with a bilateral Hoffmann sign, and fasciculation. [A] Axial T1 MTC image showed an abnormal hyperintensity involving the cortical and subcortical frontal areas including pre-central gyri and the remaining subcortical regions of the frontal lobes. [B-C] Axial and coronal FLAIR images depicted left temporal lobe atrophy with blurring of the grey/white matter differentiation (arrows). [D] Brain macroscopy confirmed atrophy in the left temporal lobe (arrow). Left hippocampus cortex showed neuronal loss as well as cytoplasmic, nuclear and extracellular UI inclusions, in addition to neuronal loss and astrogliosis (not shown).

Table 1 A summary of genetic, clinical and brain histopathology data together with the possible target of mutations in ALS and/or FTD.²⁰20. HARDY J, ROGAEVA E. MOTOR NEURON DISEASE AND FRONTOTEMPORAL DEMENTIA: SOMETIMES RELATED, SOMETIMES NOT. EXP NEUROL 2014;262 PT B: 75-83. ⁸²82. NG ASL, RADEMAKERS R, MILLER BL. FRONTOTEMPORAL DEMENTIA: A BRIDGE BETWEEN DEMENTIA AND NEUROMUSCULAR DISEASE. ANN NY ACAD SCI 2014; 1338:71-93.

In general, the inclusions are ubiquitin-positive and contain the ubiquitin binding protein p62. (ALS: Amyotrophic Lateral Sclerosis; FTD: Frontotemporal dementia; UMN: upper motor neuron; bvFTD= behavioural Frontotemporal dementia)

Table 2 Defining cognitive and behavioural subtypes in ALS.⁸³

ALS-FTD	ALS-bvFTD	ALS patient meeting either the Neary criteria or Hodge's criteria for FTD
ALS-PNFA	ALS patient meeting Neary criteria for PNFA
ALS-SD	ALS patient meeting Neary criteria for SD
Other forms	ALSbi	ALS patient meeting at least two non-overlapping supportive diagnostic features from either the Neary criteria or Hodge's criteria for FTD
ALSci	Evidence of cognitive impairment at or below the 5th percentile on at least two different tests of cognition that are sensitive to executive functioning
FTD-MND like	A neuropathological diagnosis with a primary frontotemporal lobar degeneration diagnosis with evidence of MND-type degeneration but insufficient to be classified as ALS
ALS dementia	ALS with dementia, not typical of FTD (ALS-Alzheimer, ALS-vascular dementia)
ALS-parkinsonian dementia complex	ALS concurrent with dementia and/or parkinsonianism occurring in hyperendemic foci of the Western Pacific

ALS: Amyotrophic Lateral Sclerosis; FTD: Frontotemporal Dementia; ALS-bvFTD: Amyotrophic Lateral Sclerosis and behavioural Frontotemporal Dementia; ALS-PNFA= Amyotrophic Lateral Sclerosis and Primary Non-fluent Aphasia; ALS-SD: Amyotrophic Lateral Sclerosis and Semantic Dementia; ALSbi: Amyotrophic Lateral Sclerosis with behavioural impairment; ALSci: Amyotrophic Lateral Sclerosis with cognitive impairment; MND: Motor Neuron Disease.

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Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices, Torre Norte, São Paulo, SP, Brazil, CEP 04101-000, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revistadementia@abneuro.org.br | demneuropsy@uol.com.br

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Dementia in motor neuron disease: reviewing the role of MRI in diagnosis

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[1] .Antonio José da Rocha. Santa Casa de Misericordia de São Paulo / Serviço de Diagnostico por Imagem - Rua Dr. Cesário Motta Junior, 112 - 01221-020 São Paulo SP - Brazil. E-mail: a.rocha@uol.com.br