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geri:dementia:primary-progressive-aphasia-ppa [on April 21, 2020]
geri:dementia:primary-progressive-aphasia-ppa [on November 24, 2021] (current)
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 ====== Primary Progressive Aphasia (PPA) ====== ====== Primary Progressive Aphasia (PPA) ======
 +{{INLINETOC}}
 ===== Primer ===== ===== Primer =====
-**Primary Progressive Aphasia (PPA)** is a neurological syndrome associated with certain types of dementia. ​[[geri:​dementia:​alzheimers|Alzheimer’s Disease]] ​and [[geri:​dementia:​frontotemporal|frontotemporal dementia]] ​are the most common underlying forms of dementia ​in which PPA occurs. PPA typically begins ​as gradual, subtle language deficits that progresses to a nearly complete inability to speak.+**Primary Progressive Aphasia (PPA)** is a clinically diverse ​neurological syndrome ​most commonly ​associated with [[geri:​dementia:​alzheimers|Alzheimer’s Disease]] ​or [[geri:​dementia:​frontotemporal|frontotemporal dementia]]. It can also rarely occur in [[geri:​parkinsons|Parkinson'​s]]-plus disorders. PPA typically begins ​with gradual, subtle language deficits that progresses to a nearly complete inability to speak. 
 + 
 +== Epidemiology == 
 +The age of onset is 50s to early 60s. It affects males and females equally. In terms of incidence within diagnosed dementias, [[geri:​dementia:​frontotemporal|frontotemporal dementia]] represents approximately 15% of all dementia cases, and PPA accounts for between 20 to 40% of the these patients. 
 + 
 +== Prognosis == 
 +The progression of PPA begins with asymptomatic changes in the language centres, followed by early signs of symptoms of mild cognitive impairment. The progression of non-amnestic MCI continues, until prominent aphasia is observed.[([[https://​pubmed.ncbi.nlm.nih.gov/​21573887/​|Dickerson,​ B. C. (2011). Quantitating severity and progression in primary progressive aphasia. Journal of Molecular Neuroscience,​ 45(3), 618-628.]])] The disease further progresses into mutism for most cases. Loss of independence occurs 7 years into the diagnosis in about 50% of cases. The average survival time is 7 to 10 years. After the diagnosis of PPA, patients will typically be diagnosed with a secondary syndrome (e.g. - [[geri:​dementia:​frontotemporal|frontotemporal dementia]], [[geri:​dementia:​progressive-supranuclear-palsy-psp|progressive supranculear palsy]], or [[geri:​dementia:​corticobasal-degeneration-cbd|corticobasal degeneration]]).[([[https://​pubmed.ncbi.nlm.nih.gov/​16033782/​|Kertesz,​ A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. G. (2005). The evolution and pathology of frontotemporal dementia. Brain, 128(9), 1996-2005. 
 +]])] 
 + 
 +== Risk Factors == 
 +Developmental disability is a risk factor for PPA.[([[https://​pubmed.ncbi.nlm.nih.gov/​18268195/​|Rogalski,​ E., Johnson, N., Weintraub, S., & Mesulam, M. (2008). Increased frequency of learning disability in patients with primary progressive aphasia and their first-degree relatives. Archives of neurology, 65(2), 244-248.]])]
  
 ==== Variants ==== ==== Variants ====
 There are three variants of PPA: There are three variants of PPA:
-  - Semantic (the dominant type and is present in most instances of PPA) +  - **Semantic** (the dominant type and is present in most instances of PPA) 
-  - Logopenic (LPA), due to atrophy in the left posterior temporal cortex and inferior parietal lobule +  - **Logopenic** (LPA), due to atrophy in the left posterior temporal cortex and inferior parietal lobule 
-  - Non-fluent/​agrammatic (stumbling for words, grammar is very poor)+  - **Non-fluent/​agrammatic** (stumbling for words, grammar is very poor) 
 + 
 +<WRAP group> 
 +===== Scales ===== 
 +<panel title="​PPA Scales"​ no-body="​true">​ 
 +<​mobiletable 1> 
 +^ Name                                         ^ Rater      ^ Description ​                                                                                                                                                                                                                                                                                                                                           ^ Download ​                                                     ^ 
 +^ Progressive Aphasia Symptom Severity (PASS) ​ | Clinician ​ | The PASS is a clinical instrument used to rate presence and severity of impairment in specific domains of speech and language, as opposed to making a single global language rating. The clinician make ratings on a 5 point scale from “normal” (0) to “questionable/​very mild” (0.5), “mild” (1.0), “moderate” (2.0), or “severe” (3.0) impairment.[([[https://​pubmed.ncbi.nlm.nih.gov/​21573887/​|Dickerson,​ B. C. (2011). Quantitating severity and progression in primary progressive aphasia. Journal of Molecular Neuroscience,​ 45(3), 618-628.]])] ​ | [[http://​www.nmr.mgh.harvard.edu/​~bradd/​PASS.html|Download]] ​ | 
 +</​mobiletable>​ 
 +</​panel>​ 
 + 
 +===== Diagnosis ===== 
 +<alert icon="​fa fa-arrow-circle-right fa-lg fa-fw" type="​success">​See also article: **[[neurology:​approach-aphasia|]]**</​alert>​ 
 +<WRAP group> 
 +<WRAP half column>​ 
 +The 2011 Criteria proposed by Gorno-Tempini et al are:​[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3059138/​|Gorno-Tempini,​ M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. E. E. A., ... & Manes, F. (2011). Classification of primary progressive aphasia and its variants. Neurology, WNL-0b013e31821103e6.]])] 
 + 
 +For any PPA subtype, there is ''​all''​ of: 
 +  - [[neurology:​approach-aphasia|Aphasia]] is the most prominent feature, and occurs at onset and for initial phase of disease (~2yrs) 
 +  - Causes impairment in activities of daily living (ADLs) 
 +</​WRAP>​ 
 +<WRAP half column>​ 
 +<callout type="​danger"​ title="​Exclusion Criteria"​ icon="​true">​ 
 +Alternative diagnoses should be considered if: 
 +  * If symptoms are better accounted for by other neurodegenerative,​ medical or psychiatric disorder  
 +  * If there are prominent initial memory (visual or episodic), visuoperceptual impairments,​ or behavioural disturbance within the first 2 years of language impairment 
 +</​callout>​ 
 +</​WRAP>​ 
 +</​WRAP>​ 
 + 
 +<WRAP group> 
 + 
 +<WRAP third column>​ 
 +==== Semantic ==== 
 +In the **semantic** subtype (also known as **semantic dementia** or **PPA-S**), individuals will have receptive dysfunction and loss of word meaning. There can be prosopagnosia and remote memory loss as well. Speech remains fluent. 
 + 
 +**Both** of: 
 +  - Impaired confrontation naming (i.e. - showing an object or a line drawing of an object (e.g. - a spoon) to a patient and requesting the correct verbal label for that object) 
 +  - Impaired single word comprehension 
 + 
 +At least ''​3''​ of 4: 
 +  - Impaired object knowledge 
 +  - Surface dyslexia/​dysgraphia 
 +  - Spared repetition 
 +  - Spared speech production 
 + 
 +<callout type="​tip"​ icon="​true"​ title="​Semantic is More Behavioural">​ 
 +Of the 3 subtypes of PPA, semantic dementia is associated with more behavioural changes. 
 +</​callout>​ 
 + 
 +</​WRAP>​ 
 +<WRAP third column>​ 
 +==== Logopenic ==== 
 +The **logopenic variant** (PPA-L or lvPPA) is characterized by impairment in word retrieval with phonological deficits, and impaired repetition. 
 + 
 +**Both** of: 
 +  - Impaired single word retrieval 
 +  - Impaired repetition 
 + 
 +At least ''​3''​ of 4: 
 +  - Speech errors in spontaneous speech and naming 
 +  - Spared single-word comprehension and object knowledge 
 +  - Spared motor speech 
 +  - Absence of frank agrammatism 
 + 
 +<callout type="​tip"​ icon="​true"​ title="​Digit Span is Worse">​ 
 +Individuals with lvPPA have an impaired phonological loop and are significantly worse on the repetition tasks (even compared to Alzheimer'​s),​ on tasks such as on forward and backward digit span.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC4521400/​|Meyer,​ A. M., Snider, S. F., Campbell, R. E., & Friedman, R. B. (2015). Phonological short-term memory in logopenic variant primary progressive aphasia and mild Alzheimer'​s disease. Cortex, 71, 183-189.]])] 
 +</​callout>​ 
 +</​WRAP>​ 
 + 
 +<WRAP third column>​ 
 +==== Non-fluent/​Agrammatic ==== 
 +The **Non-fluent** subtype (also know as **progressive non-fluent aphasia, PNFA**, or also called **agrammatic variant, PPA-G**) is characterized by distortion of word and sentence construction. This includes abnormal order of words (i.e. - syntax), distortion of word endings, misuse of pronouns, and a lack of grammatical words such as articles (words that define a noun as specific or unspecific, e.g. - //a, an, the//) and prepositions (these are words show the relationship between the noun and pronoun in a sentence, e.g. - //at, for, in, off, on, over, under, into, upon, onto, out of, from within//) 
 + 
 +At least ''​1''​ of 2: 
 +  - Agrammatism 
 +  - Apraxia of speech 
 +At least ''​2''​ of 3: 
 +  - Impaired complex comprehension 
 +  - Spared single-word comprehension 
 +  - Spared object knowledge 
 + 
 +<callout title="​Neuroanatomy Findings"​ type="​tip"​ icon="​true">​ 
 +In patients with PNFA, there is asymmetric perisylvian and anterior insular atrophy with the dominant language hemisphere most affected (usually the left hemisphere, assuming the individual is right-handed).[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC2916644/​|Rabinovici,​ G. D., & Miller, B. L. (2010). Frontotemporal lobar degeneration. CNS drugs, 24(5), 375-398.]])] 
 +</​callout>​ 
 +</​WRAP>​ 
 +</​WRAP>​ 
 + 
 + 
 + 
 +==== Symptoms ==== 
 +<alert type="​info"​ icon="​fa fa-book fa-lg fa-fw">​See also: **[[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3753029/​|Modirrousta,​ M. et al. (2013). Neuropsychiatric symptoms in primary progressive aphasia: phenomenology,​ pathophysiology,​ and approach to assessment and treatment. Neurodegenerative disease management, 3(2), 133-146.]]**</​alert>​ 
 + 
 +Depression, anxiety, and dysphoria are the most common symptoms across all subtypes of PPA.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3753029/​|Modirrousta,​ M., Price, B. H., & Dickerson, B. C. (2013). Neuropsychiatric symptoms in primary progressive aphasia: phenomenology,​ pathophysiology,​ and approach to assessment and treatment. Neurodegenerative disease management, 3(2), 133-146.]])] Commonly, patients have also have [[teaching:​apathy|apathy]]. Changes in eating behaviors are more evident in PPA patients, including hyperorality. Psychotic symptoms in PPA are rare. Disinhibition in patients should make the clinician think of an underlying FTD pathology rather than Alzheimer'​s pathology for the PPA. 
 + 
 + 
 +===== Comparison ===== 
 +<panel type="​info"​ title="​PPA subtypes"​ subtitle=""​ no-body="​true"​ footer="">​ 
 +<​mobiletable 1> 
 +^ Subtype ​                      ^ Semantic (PPA-S) ​                                                                                                                ^ Logopenic (PPA-L) ​                                                                                                                                       ^ Agrammatic (PPA-G) ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                    ^ 
 +^ Other abbreviations or names  | • Semantic dementia (SD) \\ • Semantic variant PPA (svPPA) ​                                                                      | • Logopenic variant PPA (lvPPA) ​                                                                                                                         | • Non-fluent variant PPA (nfvPPA) \\ •  Progressive non-fluent aphasia (PNFA) ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                         | 
 +^ Grammar ​                      | **N**                                                                                                                            | **N**                                                                                                                                                    | //​Imp// ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                               | 
 +^ Repetition ​                   | **N**                                                                                                                            | //​Imp// ​                                                                                                                                                 | //​Imp// ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                               | 
 +^ Single word comprehension ​    | //​Imp// ​                                                                                                                         | **N**                                                                                                                                                    | **N**                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                  | 
 +^ Naming ​                       | //Imp// (due to impaired word-retrieval and comprehension) ​                                                                      | //Imp// (due to impaired word-retrieval and anomia) ​                                                                                                     | **N** or //​Imp// ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                      | 
 +^ Speech and Fluency ​           | Speech is vague but remains fluent, with circumlocutions and semantic paraphasias ​                                               | May appear normal during small talk. Word retrieval pauses leads to some degree of loss of fluency. Circumlocution and phonemic paraphasias are common. ​ | Effortful and apraxic, word-finding hesitations. Abnormal order of words, distortion of word endings, misuse of pronouns, and a lack of grammatical words such as (e.g. - //a, the//), or prepositions (e.g. - //at, for, on, into//​) ​                                                                                                                                                                                                                                                                                                                 | 
 +^ Sample speech ​                | Saying “clock” instead of “watch” when asked to name a wristwatch ​                                                               | Saying "baby flitter” instead of “baby sitter” ​                                                                                                          | Syntax and speech is abnormal, with poor use of tense and modifiers ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                   | 
 +^ Neuroimaging ​                 | Atrophy in the anterior temporal lobe                                                                                            | Atrophy in posterior temporoparietal part of language network ​                                                                                           | Atrophy in left inferior frontal gyrus                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                 | 
 +^ Common Pathology ​             | FTD with TDP-43 type C (80-90% cases) ​                                                                                           | Alzheimer'​s ​                                                                                                                                             | FTD with tauopathy (60% of cases) ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                     | 
 +^ Rarer Pathology ​              | Alzheimer'​s ​                                                                                                                     | • FTD with TDP-43 type A \\ • FTD with tau                                                                                                               | •  FTD with TDP-43 type A \\ •  Alzheimer'​s ​                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                           | 
 +^ Clinical progression ​         | Cases may progress into symptoms characteristic of behavioural-variant [[geri:​dementia:​frontotemporal|frontotemporal dementia]] ​ | There is typically a mix of aphasia, and memory difficulties,​ characteristic of the predominant Alzhemier'​s pathology. ​                                  | Parkinsonism most commonly occurs in the non-fluent subtype, with a [[geri:​dementia:​corticobasal-degeneration-cbd|corticobasal]] or [[geri:​dementia:​progressive-supranuclear-palsy-psp|progressive supranuclear palsy (PSP)]] etiology being the most likely. In both cases, the underlying pathology is a tauopathy.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC3748791/​|Doherty,​ K. M., Rohrer, J. D., Lees, A. J., Holton, J. L., & Warren, J. (2013). Primary progressive aphasia with parkinsonism. Movement Disorders, 28(6), 741-746.]])] ​ | 
 +</​mobiletable>​ 
 +</​panel>​ 
 + 
 +===== Pathophysiology ===== 
 +<alert type="​info"​ icon="​fa fa-book fa-lg fa-fw">​See also: **[[https://​pubmed.ncbi.nlm.nih.gov/​25179257/​|Mesulam,​ M. M. et al. (2014). Primary progressive aphasia and the evolving neurology of the language network. Nature Reviews Neurology, 10(10), 554.]]**</​alert>​ 
 + 
 +==== Genetics === 
 +The vast majority of PPA cases are sporadic. Of the genes that have been identified, there is variability in transmission of these genes. Risk factor genes include GRN (granulin), MAPT (tau), C9ORF72 (protein C9orf72), and PSEN1 (presenilin1). 
 + 
 +==== Pathology ==== 
 +Since PPA is a syndromic diagnosis, its' pathophysiological etiology can be divided into one of two underlying pathophysiological processes:​[([[https://​pubmed.ncbi.nlm.nih.gov/​21644037/​|Mackenzie,​ I. R., Neumann, M., Baborie, A., Sampathu, D. M., Du Plessis, D., Jaros, E., ... & Lee, V. M. (2011). A harmonized classification system for FTLD-TDP pathology. Acta neuropathologica,​ 122(1), 111-113.]])] 
 +  * [[geri:​dementia:​alzheimers|Alzheimer'​s]] pathology (40-45% of cases) 
 +  * [[geri:​dementia:​frontotemporal|Frontotemporal dementia]] pathology (55-60% of cases) 
 +    * 50% is TDP-43 pathology 
 +    * 50% is tau pathology 
 + 
 +For each PPA subtype, the most common neuropathologies are:​[([[https://​pubmed.ncbi.nlm.nih.gov/​21644037/​|Mackenzie,​ I. R., Neumann, M., Baborie, A., Sampathu, D. M., Du Plessis, D., Jaros, E., ... & Lee, V. M. (2011). A harmonized classification system for FTLD-TDP pathology. Acta neuropathologica,​ 122(1), 111-113.]])] 
 +  * **Semantic**:​ FTD with TDP-43 type C (80-90% of cases) 
 +  * **Logopenic**:​ Alzheimer'​s  
 +  * **Non-fluent (agrammatic)**:​ FTD with tau (60% of cases) 
 + 
 +===== Differential Diagnosis ===== 
 +<WRAP group> 
 +<WRAP half column>​ 
 +  * **Frontal-subcortical dementias** can present with anomia due to slowed word retrieval in the setting of fronto-subcortical dysfunction,​ this includes:​[([[https://​pubmed.ncbi.nlm.nih.gov/​18332839/​|Bonelli,​ R. M., & Cummings, J. L. (2008). Frontal-subcortical dementias. The neurologist,​ 14(2), 100-107.]])] 
 +    * [[cl:​huntingtons-disease|Huntington chorea]] 
 +    * [[geri:​dementia:​parkinsons|Parkinson disease dementia]] 
 +    * [[geri:​dementia:​progressive-supranuclear-palsy-psp|Progressive supranuclear palsy]] 
 +    * Thalamic degeneration 
 +    * Subcortical [[geri:​dementia:​vascular|vascular dementia]] 
 +    * [[cl:​multiple-sclerosis|Multiple sclerosis]] 
 +    * Acquired immunodeficiency syndrome dementia complex 
 +    * [[mood:​1-depression:​geriatric|Depressive pseudodementia (geriatric depression)]] 
 +    * Spinocerebellar degenerative syndromes 
 +    * Hallervorden–Spatz disease 
 +    * Choreoacanthocytosis 
 +</​WRAP>​ 
 +<WRAP half column>​ 
 +  * Idiopathic basal ganglia calcification 
 +  * Guamanian parkinsonism–dementia complex 
 +  * [[geri:​dementia:​corticobasal-degeneration-cbd|Corticobasal degeneration]] 
 +  * [[geri:​dementia:​multiple-system-atrophy-msa|Multiple system atrophy]] 
 +  * [[cl:​wilsons-disease|Wilson'​s disease]] 
 +  * Metachromatic leukodystrophy 
 +  * Adrenoleukodystrophy 
 +  * [[cl:​hypercalcemia-hyperparathyroidism|Hypoparathyroidism]] 
 +  * Ssarcoidosis 
 +  * Other CNS inflammatory disorders 
 +</​WRAP>​ 
 +</​WRAP>​ 
 + 
 + 
 +===== Investigations ===== 
 +==== Cognitive Testing ==== 
 +<alert icon="​fa fa-arrow-circle-right fa-lg fa-fw" type="​success">​See main article: **[[:​cognitive-testing|]]**</​alert>​ 
 + 
 +Since PPA has disproportionate language impairment compared to other dementias, cognitive testing scores (especially on language-sensitive tests such as the [[cognitive-testing:​moca|Montreal Cognitive Assessment]]) can appear significantly worse compared to the patient'​s actual functional ability and stage of dementia.  
 + 
 +==== Neuroimaging ==== 
 +  * PPA typically has a distinct asymmetric (language-dominant) perisylvian atrophy pattern on MRI. MRI findings for each subtype include:​[([[https://​pubmed.ncbi.nlm.nih.gov/​21573887/​|Dickerson,​ B. C. (2011). Quantitating severity and progression in primary progressive aphasia. Journal of Molecular Neuroscience,​ 45(3), 618-628.]])] 
 +    * PPA-G, peak atrophy in left inferior frontal gyrus 
 +    * PPA-S, peak atrophy in the anterior frontal lobe 
 +    * PPA-L, peak atrophy in the temporoparietal junction 
 + 
 +===== Treatment ===== 
 +==== Non-pharmacological ==== 
 +  * Speech language pathology (SLP) involvement is important to preserve function and improve quality of life.[([[https://​pubmed.ncbi.nlm.nih.gov/​23611353/​|Kortte,​ K. B., & Rogalski, E. J. (2013). Behavioural interventions for enhancing life participation in behavioural variant frontotemporal dementia and primary progressive aphasia. International Review of Psychiatry, 25(2), 237-245.]])]. SLPs can teach patients to maximize communication through the use of nonverbal techniques. Intensive language therapy can also occur.[([[https://​pubmed.ncbi.nlm.nih.gov/​27297727/​|Jokel,​ R., Kielar, A., Anderson, N. D., Black, S. E., Rochon, E., Graham, S., ... & Tang-Wai, D. F. (2016). Behavioural and neuroimaging changes after naming therapy for semantic variant primary progressive aphasia. Neuropsychologia,​ 89, 191-216.]])] 
 +  * Aphasia identification cards and assistive devices (e.g. - iPads) 
 +  * Exercise can improve cognition, mood, and overall health in all dementias[([[https://​pubmed.ncbi.nlm.nih.gov/​23582750/​|Cheng,​ S. T., Chow, P. K., Song, Y. Q., Edwin, C. S., Chan, A. C., Lee, T. M., & Lam, J. H. (2014). Mental and physical activities delay cognitive decline in older persons with dementia. The American Journal of Geriatric Psychiatry, 22(1), 63-74.]])] 
 +  * Addressing caregiver burden/​burnout,​ safety (i.e. - wandering, stove/taps) is important, family members should be offered support groups for family living loved ones with dementia 
 + 
 +==== Pharmacological ==== 
 +All current medications for PPA are off-label treatments. There are no treatments that can stop or alter the course of disease. 
 +  * [[meds:​dementia:​home|Cholinesterase inhibitors]] can be trialed if the underlying pathology is thought to due to Alzheimer'​s pathology (i.e. - logopenic PPA). 
 +    * [[meds:​dementia:​galantamine|Galantamine]] has been shown to help with stabilizing language function[([[https://​pubmed.ncbi.nlm.nih.gov/​18196898/​|Kertesz,​ A., Morlog, D., Light, M., Blair, M., Davidson, W., Jesso, S., & Brashear, R. (2008). Galantamine in frontotemporal dementia and primary progressive aphasia. Dementia and geriatric cognitive disorders, 25(2), 178-185.]])] 
 +    * If the pathology is thought to be due to behavioural variant FTD (bvFTD), then acetylcholinesterase inhibitors may worsen symptoms (as is seen in FTD), and should not be used![([[https://​pubmed.ncbi.nlm.nih.gov/​24026732/​|Arciniegas,​ D. B., & Anderson, C. A. (2013). Donepezil-induced confusional state in a patient with autopsy-proven behavioral-variant frontotemporal dementia. The Journal of Neuropsychiatry and Clinical Neurosciences,​ 25(3), E25-E26.]])][([[https://​pubmed.ncbi.nlm.nih.gov/​23551349/​|Kimura,​ T., & Takamatsu, J. (2013). Pilot study of pharmacological treatment for frontotemporal dementia: risk of donepezil treatment for behavioral and psychological symptoms. Geriatrics & Gerontology International,​ 13(2), 506-507.]])] 
 +  * There is no evidence for the use of [[meds:​dementia:​memantine|memantine]],​ and again may possibly worsen behavioural symptoms.[([[https://​pubmed.ncbi.nlm.nih.gov/​23290598/​|Boxer,​ A. L., Knopman, D. S., Kaufer, D. I., Grossman, M., Onyike, C., Graf-Radford,​ N., ... & Koestler, M. (2013). Memantine in patients with frontotemporal lobar degeneration:​ a multicentre,​ randomised, double-blind,​ placebo-controlled trial. The Lancet Neurology, 12(2), 149-156.]])]
  
-===== Diagnostic Criteria ===== +Treatment of [[geri:​dementia:​1-bpsd|behavioural and psychological symptoms]] for PPA (due to bvFTD) include: 
-  * [[https://www.ncbi.nlm.nih.gov/​pmc/articles/PMC3059138/|Gorno-TempiniM. L., HillisA. E., WeintraubS., KerteszA., MendezM., Cappa, S. E. EA., ... & ManesF. (2011). Classification ​of primary progressive aphasia ​and its variants. Neurology, ​WNL-0b013e31821103e6.]]+  * [[meds:​antidepressants:​home|Antidepressants]] 
 +    * [[meds:​antidepressants:​ssri:​sertraline|Sertraline]],​ [[meds:​antidepressants:​ssri:​citalopram|citalopram]],​ [[meds:​antidepressants:​sari:​trazodone|trazodone]][([[https://pubmed.ncbi.nlm.nih.gov/​12392261/|Chow, T. W., & Mendez, M. F. (2002). Goals in symptomatic pharmacologic management of frontotemporal lobar degeneration. American Journal of Alzheimer'​s Disease & Other Dementias®,​ 17(5), 267-272.]])][([[https:​//​pubmed.ncbi.nlm.nih.gov/​21878805/|Herrmann, N., Black, S. E., Chow, T., Cappell, J., Tang-WaiD. F., & Lanctôt, K. L. (2012). Serotonergic function and treatment of behavioral and psychological symptoms of frontotemporal dementia. The American Journal of Geriatric Psychiatry20(9)789-797.]])] 
 +  * [[meds:​antipsychotics:​home|Antipsychotics]] 
 +    * [[meds:​antipsychotics:​second-gen-atypical:​6-quetiapine|Quetiapine]],​ [[meds:​antipsychotics:​second-gen-atypical:​1-risperidone|risperidone]],​ and [[meds:​antipsychotics:​second-gen-atypical:​3-aripiprazole|aripiprazole]] 
 +  * [[meds:​mood-stabilizers-anticonvulsants:​home|Mood stabilizers]] 
 +    * [[meds:​mood-stabilizers-anticonvulsants:​1-valproic-divalproex|Valproic acid]], [[meds:​alcohol:​topiramate|topiramate]],​ and [[meds:​mood-stabilizers-anticonvulsants:​carbamazepine|carbamazepine]] 
 +  * Other investigational treatments include the use of oxytocin to decrease apathy and improve empathy.[([[https://​www.ncbi.nlm.nih.gov/​pmc/​articles/​PMC4336088/​|Finger, ​E. C., MacKinleyJ., BlairM., OliverL. D., Jesso, S., Tartaglia, MC., ... & MitchellD. G. (2015). Oxytocin for frontotemporal dementia: a randomized dose-finding study of safety ​and tolerability. Neurology, ​84(2), 174-181.]])]
  
 +===== Resources =====
 +== For Patients ==
 +  * [[https://​memory.ucsf.edu/​dementia/​primary-progressive-aphasia|UCSF:​ Primary Progressive Aphasia]]
 +  * [[https://​www.youtube.com/​watch?​v=rAikhxAHBOQ&​ab_channel=TheAFTD|YouTube:​ AFTD Webinar: What You Should Know about Primary Progressive Aphasia (PPA)]]