Motor neuron disease associated with non‐fluent rapidly progressive aphasia: case report and review of the literature

Abstract

The superimposed clinical features of motor neuron disease (MND) and frontotemporal lobar degeneration (FTLD) comprise a rare neurological overlap syndrome that represents a diagnostic challenge to neurologists. Currently, FTLD-MND is considered a distinct entity and its clinicopathological basis has recently been reviewed. Our aim is to present a patient with MND and non-fluent rapidly progressive aphasia with clinical, imaging and histopathological correlation, as well as a brief review of the literature. We demonstrated the selective corticospinal tract (CST) and temporal lobe involvement using T1 spin-echo with an additional magnetization transfer contrast pulse on resonance (T1 SE/MTC) and FLAIR MR sequences in our patient, with further clinical and histopathological correlation. To the best of our knowledge, there is no description about the use of these particular MR sequences in the evaluation of FTLD-MND patients.

Kana versus Kanji in Amyotrophic Lateral Sclerosis: A Clinicoradiological Study of Writing Errors

“…Writing errors in ALS patients have been described as 'spelling errors', 'paragraphia or paraphasia in written language' and 'syntactic errors' in previous studies in alphabetical languages [14,15,17,19,21,23,24,27] , but we classified Japanese writing errors into 'omission', 'substitution', 'insertion' and 'syntactic errors' in the current study. To date, clinicoradiological relationships with writing errors have not been studied in detail, and the characteristics and clinical significance of these errors remain unclear.…”

“…In the Japanese writing system, the dual-route hypothesis for kana and kanji is supported by observations made exclusively in patients with cerebrovascular disease [29][30][31][32][33][34] . There is little information on the characteristics of writing errors, including dissociation between kana and kanji, in ALS patients [21,25,27] , but findings are available in patients with FTLD, PNFA and SD, which have clinical phenotypes distinct from FTD [1][2][3] . PNFA is a disorder of expressive language, characterized by effortful speech production, phonologic and grammatical errors, and word retrieval difficulties, while understanding of word meaning is relatively well preserved [1][2][3] .…”

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

“…Our pathological findings also predominated in the same sites depicted by these MRI sequences. 66 While T1 MTC is able to demonstrate motor and extra-motor involvement in both CST and frontal lobes and is positively coincident to brain injury, as reflected in the UI distribution, T2/FLAIR images are useful for demonstrating subcortical gliosis, as confirmed by histopathological analysis ( Figure 2 ).…”

“…58 , 63 , 64 Structural MR sequences, including FLAIR and 3D acquisitions, are also recommended when extra-motor involvement is suspected, considering the MND/FTD spectrum. 66 , 68 Further investigations using structural and nonconventional techniques are recommended to identify MR features in MND-FTD patients and to correlate in vivo abnormalities with neuropathological diagnostic criteria. Future genetic, clinicopathological and biochemical results remain necessary for fuller comprehension of the MND-FTD spectrum, while determining the imaging correlation amongst clinical, imaging, and histopathologic features in this overlapping syndrome is highly desirable.…”