Journal of the Neurological Sciences 347 (2014) 387–388

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Letter to the Editor Primary progressive aphasia with occipital impairment Keywords: Primary progressive aphasia Posterior cortical atrophy Dementia Amyloid

battery scoring 18 over 20 in position discrimination, 8 over 10 in number location; 9 over 20 in shape decision and 12 over 20 in progressive silhouettes) (5–7) A 18F-FDG-PET performed 6 months after clinical onset showed left occipito-temporo-parietal hypometabolism. He also presented a diffuse retention of amyloid tracer 18F-Florbetapir, with a loss of differentiation between white and gray matter (Fig. 1). Discussion

Primary progressive aphasia (PPA) is a clinical entity characterized by a neurodegenerative language disorder. The current classification in three variants of PPA has improved the ability to predict the pathological substrate (1,2). Wicklund et al. have recently described two clinical cases of progressive aphasia with left occipitotemporal hypometabolism, suggesting an overlapping form with posterior cortical atrophy (PCA), a subtype of focal onset of Alzheimer's disease (3). We present two cases of progressive aphasia with left parieto-temporo-occipital hypometabolism in fluorodeoxyglucose positron emission tomography (18F-FDG-PET), and amyloid deposition in PET imaging with 18F-Florbetapir. These two cases support the existence of a subtype of primary progressive aphasia with associated left occipital lobe impairment. In this article, we discuss the clinical significance of this variant. The first case was a right-handed 69-year-old woman with below basic literacy level. She developed a word finding difficulty 3 years ago, with no other associated symptomatology. Her speech had a qualitative reduction in fluency, with anomia and phonemic paraphasias, and a disturbance in the repetition of long phrases and nonwords. Her cognitive symptoms progressed slowly, and she developed global dementia over 2 years later. During this period she never presented Parkinsonism, hallucinations or REM behavior disorder. Moreover, she showed a normal uptake of 123I-ioflupane using single-photon emission computed tomography. A 18F-FDG-PET performed 3 years after clinical onset showed left occipito-temporo-parietal hypometabolism. 5 years after clinical onset, a second 18F-FDG-PET showed a more pronounced left occipitotemporo-parietal hypometabolism, and PET imaging with 18F-Florbetapir revealed diffused amyloid deposition (Fig. 1). The second case was a right-handed 70-year-old man, nonprofessional painter. He presented with a 6-month history of wordfinding difficulty, without any other symptom and keeping his painting skills unimpaired. On the neuropsychological examination he reached 29 over 30 in the Minimental State Examination and 73 over 100 in the Addenbrooke's Cognitive Examination (4). The language examination showed anomia in confrontation naming (Boston Naming Test 26 over 60; 34 phonemic cues were provided, and the patient correctly answered in 22), without agramatism and unimpaired repetition and semantic knowledge. Visuospatial and visuoperceptive skills were impaired (judgment of line orientation 13 over 30; visual object and space perception 0022-510X/© 2014 Elsevier B.V. All rights reserved.

We reported two patients with progressive aphasia, the first case fulfilled the criteria for the logopenic variant of PPA (1), whereas the second one presented a nominal aphasia. At the time of 18F-FDG-PET performance, neither showed other functional or cognitive impairment besides language (except discrete visuospatial and visuoperceptive deficits in case 2), but both showed left hemispheric hypometabolism with left occipital involvement. The PET pattern of hypometabolism exposes some grade of overlapping between PPA logopenic variant (left parieto-temporal) and PCA (bilateral occipitoparietal). The presence of occipital hypometabolism in patients with Alzheimer's disease has been considered suggestive of Lewy Body Dementia (LBD), even useful as an early marker (8). Nevertheless, none of the patients developed during their entire follow up symptoms related with LBD, such as REM behavior disorder or Parkinsonism. Both patients exhibited retention of amyloid tracer 18F-florbetapirPET, in the same way as the previously communicated patients did in PiB-PET (3). This indicates the presence of moderate to frequent neuritic plaques (9). These findings suggest a link between the presence of occipital hypometabolism in PPA patients and Alzheimer pathology. Therefore, the association between Alzheimer's disease and PPA patients with occipital hypometabolism could be higher than the one found with the logopenic variant, in which several non-Alzheimer patients have been reported (10,11). Given the presence of visuospatial impairment and occipital hypometabolism, these patients could be considered as an overlapping entity between PPA and PCA. In fact, some works about PCA deal about the linguistic profile of PCA, usually logopenic-like, with a variable prevalence (12,13). Notwithstanding, visuospatial deficits were subclinical in the reported patients, so they should be classified inside the PPA group. This suggests another possible phenotype in the logopenic variant group (14). In conclusion, the reported cases suggest the existence of a PPA subgroup with occipital involvement in functional neuroimaging and subclinical visuospatial impairment. The left occipital involvement in PPA could be a specific marker of Alzheimer pathology, although data from larger clinical series are necessary to confirm this finding. Conflict of interest The authors declare no conflict of interests.


Letter to the Editor


and 18F-florbetapir PET of the paents





Fig. 1. A) Case 1. Parieto-temporo-occipital hypometabolism is observed in FDG-PET (A1) as well as amyloid deposition in florbetapir-PET (A2). B). Case 2. Parieto-temporo-occipital hypometabolism in FDG-PET (B1), with amyloid deposition in florbetapir-PET (B2).

References [1] Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006–14. [2] Matias-Guiu JA, García-Ramos R. Primary progressive aphasia: from syndrome to disease. Neurologia 2013;28:366–74. [3] Wicklund MR, Duffy JR, Strand EA, et al. Aphasia with left occipitotemporal hypometabolism: a novel presentation of posterior cortical atrophy? J Clin Neurosci 2013;20:1237–40. [4] Sarasola D, de Luján-Calcagno M, Sabe L, et al. Validity of the Spanish version of the Addenbrooke's Cognitive Examination for the diagnosis of dementia an to differentiate Alzheimer's disease and frontotemporal dementia. Rev Neurol 2005;41:717–21. [5] Kaplan EF, Goodglass H, Weintraub S. The Boston Naming Test. Philadelphia: Lippincot Williams & Wilkins; 2001. [6] Benton A, Hamsher K, Varney NR, et al. Contributions to neuropsychological assessment: a clinical manual. New York: Oxford University Press; 1983. [7] Warrington EK, James M. The visual object and space perception battery. Bury St Edmunds, Suffolk, England: Thames Valley Test Company; 1991. [8] Minoshima S, Foster NL, Anders AF, et al. Alzheimer's disease versus dementia with lewy body: cerebral metabolic distinction with autopsy confirmation. Ann Neurol 2001;50:358–65. [9] Clark CM, Pontecorvo MJ, Beach TG, et al. Cerebral PET with florbetapir compared with neuropathology at autopsy for detection of neuritic amyloid B-plaques: a prospective cohort study. Lancet Neurol 2012;11:669–78. [10] Grossman M. Primary progressive aphasia: clinicopathological correlations. Nat Rev Neurol 2010;6:88–97. [11] Machulda MM, Whitwell JL, Duffy JR, et al. Identification of an atypical variant of logopenic progressive aphasia. Brain Lang 2013;127:139–44. [12] Magnin E, Sylvestre G, Lenoir F, et al. Logopenic syndrome in posterior cortical atrophy. J Neurol 2013;260:528–33. [13] Crutch SJ, Lehmann M, Warren JD, et al. The language profile of posterior cortical atrophy. J Neurol Neurosurg Psychiatry 2013;84:460–6. [14] Rohrer JD, Crutch SJ, Warrington EK. Progranulin-associated primary progressive aphasia: a distinct phenotype. Neuropsychologia 2010;48: 288–97.

David García-Azorín Jordi A. Matías-Guiu* Department of Neurology, Hospital Clínico San Carlos, Universidad Complutense, Health Research Institute “San Carlos”, Madrid, Spain *Corresponding author at: Department of Neurology, Hospital Clinico San Carlos, Universidad Complutense, Prof. Martin Lagos St. 28040 Madrid Spain. Tel./fax: +34 913303511. E-mail address: [email protected] (J. A. Matias-Guiu). María Nieves Cabrera-Martín Department of Nuclear Medicine, Hospital Clínico San Carlos, Universidad Complutense, Health Research Institute “San Carlos”, Madrid, Spain Marta Fernández-Matarrubia Teresa Moreno-Ramos Department of Neurology, Hospital Clínico San Carlos, Universidad Complutense, Health Research Institute “San Carlos”, Madrid, Spain José Luis Carreras Department of Nuclear Medicine, Hospital Clínico San Carlos, Universidad Complutense, Health Research Institute “San Carlos”, Madrid, Spain Jorge Matías-Guiu Department of Neurology, Hospital Clínico San Carlos, Universidad Complutense, Health Research Institute “San Carlos”, Madrid, Spain 10 August 2014

Primary progressive aphasia with occipital impairment.

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