This is not like my mum used to be Different faces of Fronto-Temporal Lobe Dementia

1. This is not like my mum used to beDifferent faces of Fronto-Temporal Lobe Dementia Snezana Mitrovic Tosovic Kingsley Mortimer Unit 8th National NZPOPs Auckland February 2007

2. Frontal Lobes Receive input from the thalamus, limbic system, hypothalamus, and connections from other lobes making it a �control centre�. Frontal lobes contain the following specialised regions: Motor cortex: governs fine motor control Brocca�s area: governs language production Prefrontal cortex: governs planning, organisation, the monitoring of recent events, the probably outcome of actions and the emotional value of such actions, decision making, emotional regulation

3. Fronto-temporal dementia: overview of the syndrome Demographic features: Pre-senile onset (most commonly between the age of 45-60; range of onset 21-75) Family history in 50% of cases Equal sex incidence Median illness duration 8 years (range 2-20 years) (Snowden&Neary) Prevalence: The prevalence of dementia increases with age from 1% at age 60 to about 40% at age 85. Four types of dementia--account for 90% of all cases. FTD accounts for 20% of pre-senile dementias (Brun,1987,Neary,1988)

4. Frontotemporal lobe degenerationA consensus on clinical diagnostic criteria Clinical Diagnostic features of Frontotemporal dementia: Core diagnostic features A �insidious onset B-early decline in social interpersonal conduct C � early impairment in personal conduct D �early emotional blunting E � early loss of insight Supportive diagnostic features: A �Behavioural disorder (decline in personal hygiene and grooming, mental rigidity, distractibility and impersistence, hyperorality, perseverative and stereotyped behaviour, utilization behaviour) B � Speech and language (aspontaneity and economy of speech, perseveration, echolalia, mutism)

5. Frontotemporal lobe degenerationA consensus on clinical diagnostic criteria, cont. Physical signs ( primitive reflexes, incontinence, akinesia, rigidity, and tremor, low and labile blood pressure). Investigations: Neuropsychology (significant impairement on frontal lobe tests in the absence of severe amnesia, aphasia or perceptuospatial disorder) Brain imaging (predominant frontal and anterior temporal abnormality Clinical diagnostic features of progressive nonfluent aphasia Core Features: A �Insidious onset B � Nonfluent spontaneous speech with at least one of the following: agrammatism, phonemic paraphasia, anomia

6. Frontotemporal lobe degenerationA consensus on clinical diagnostic criteria, cont. Supportive diagnostic features: A � Speech and language (impaired repetition, stuttering, alexia, early perseveration of word meaning, late � mutism) B � Behaviour ( early � preservation of social skills, late � behavioural changes as in FTD) C � Physical signs � late as in FTD Investigations: Neuropsychology � nonfluent aphasia in absence of severe amnesia or perceptuospatial disorder Brain imaging � asymetric abnormality predominantly affecting dominant hemisphere

7. Frontotemporal lobe degenerationA consensus on clinical diagnostic criteria, cont. Clinical diagnostic features of semantic aphasia Core diagnostic features: A � insidious onset B � Loss of word meaning manifested by impaired naming and comprehension, semantic paraphasias C � Perceptual diosorders ( prospagnosia and associative agnosia) D � preserved perceptual matching and drawing reproduction, single word repetition, ability to read loudly and write to dictation). Supportive diagnostic features: A - Speech and language (idiosyncratic use of words, surface dyslexia and dysgraphia, preserved calculation) B � Behaviour (loss of sympathy and empathy, narrowed preoccupations) C � physical signs (absent or late primitive reflexes, rigidity, tremor) Investigations: Neuropsychology - semantic loss �comprehension and naming, face and object recognition. Brain imaging � predominant anterior temporal abnormality Neary et.al.1998.

8. Case BH, 67 years old Presentation: Reported by family: Recent memory impaired, constantly talking about distant past Deterioration in personal hygiene, previously meticulous in her appearance Unable to cook even simple things � cracked eggs open onto cold element, used an egg beater to mix water in the sink when she was believing she was making a cake, boiled the cattle without a water Chain smoker Preoccupied by food � would use hands to shove large amounts of food in her mouth, occasionally chokes and brings it back up, sometimes would not swallow food completely before spitting it up Started to be verbally abusive On interview denied all the above symptoms On the word repetitive, perseverating on single words and ideas, Preoccupied with one idea at the time, difficult to distract Socially inappropriate (leave the toilet door open), undress on her way to toilet Pacing constantly Moderately dependent in her ADLs Affect labile, shallow

9. CASE BH History of presenting problems No psychiatric problems until 9-10 years Started to drink very heavily and drank for 7 years (prior to this she was a social drinker) Medical problems involve hearing deficits, ongoing weight loss, history of B12 deficiency Relevant Investigations MMSE in August 2006 25/30 CT head January 2007 - reported no abnormalities MRI head January 2007 � reported no abnormalities Medication at the time of assessment - thiamine

10. Neuropsychological Assessment FrSBe � Frontal Systems Behavioural Scale SCIP Severe Cognitive Impairment Profile WAIS III WMS III Logical Memory I and II Word List I and II Family Pictures I and II EXIT Background information Born Fijian, came to New Zealand at the age of 7 � English first language Finished high school Married with four children, two living locally, husband and children very supportive Professional background: initially worked in modeling, then as a machinist Right handed Premorbidly: meticulous, polite, gentle

11. Neuropsychological Assessment Co-operative with assessment, with fluctuating level of interest and motivation, getting distressed and tearful when could not perform, quickly re-storing mood FrSBe � family rating �completed by son who is living with parents at present � Measures: apathy, disinhibition and executive dysfunction before the illness and current- significant increase on all three dimensions � increase in disexecutive dysfunction being themost prominent SCIP � too easy WAIS III Picture completion � patchy performance, needed to be reminded of task requirements (SS 3) Vocabulary � poverty of language, perseveration, some concrete answers (SS 7) Digit Symbol-Coding � (SS 4) Similarities � switched to differences, concrete answers (e.g. fork-spoon � have handles, boat - car have steering wheel) SS -4 Block Design � SS 4, did not manage to shift from 3-dimensional to 2-dimensional model

12. Neuropsychological Assessment Arithmetic � SS 4 Matrix Reasoning SS 7 Digit Span SS 9 � forward 7 digits, backward 3 digits Information � SS 8 Picture Arrangement SS 7 � lack of attention for details, misinterpret ting some details (e.g. dog wants something) difficulty in sequencing events, correct description of pictures but does not recognize that they don�t fit into story, does not attempt to correct herself Comprehension SS 6 � some answers overly general, not able to give meaning to proverbs WMS III Logical Memory I � SS 4; II-SS 1 Family Pictures I � SS 3; II � SS3 Word List: 1st recall � 2 words, Total recall I� SS 10 II- 13 List B � 7 words EXIT � 25/50 evidence of utilization behavior, absent primitive reflexes,

13. Neuropsychological Assessment Attention Processing speed Language Memory Visuo spatial constructional Exexutive functioning

14. Case EB, 62 years old Presentation Depressed mood, anxious and �stressed�, �unnerved� by small thing Emotional and tearful Poor sleep and appetite Believes she had done � bad things� during her life and ruminating about these Some paranoid ideas Decline in memory and functioning (worsening in particular in last 3 months) could not operate washing machine or vacuum cleaner, leaves herself messages all over the place and then gets confused with all these messages Preoccupied with some traumatic events, happened many years ago (40 years) ON interview: Perplexed Anxious, Some psychomotor retardation, Mood dysphonic, Affect restricted Perseverative, Loosened association and tangential replies

15. Case EB History of the problem Cognitive and functional decline noticed in last year but more pronounced in last three months History of depressed mood spanning from her early 20�s In her 40�s became more religious Further change occurred when EB went to India (10 years ago), Daughter described her mother�s behavior being out of her character when she visited her in 2000. Personal history: Born in New Zealand, the oldest of 4, Finished high school, discontinued as needed to help to her family Vague historian, said could not remember Has one daughter, was briefly married two times Father developed dementia in his early sixties On the ward: Mood up and down, still feeling guilty for past �errors� Describes her thinking as �muddled� Continually struggling with everyday tasks (e.g.using shower)� distressed about this Attended ot activities � reported difficulty to participate in different word puzzles

16. Neuropsychological Assessment Relevant investigations MMSE in January 2007 20/30 CT head January 2007 � reported normal MRI head January 2007 � reported normal Medications At the time of assessment: 20 mg Citalopram and 1,0 mg Risperidone Medical background Nil of note Tests administered: FrSBe � Frontal Systems Behavioural Scale SCIP Severe Cognitive Impairment Profile WAIS III WMS III Logical Memory I and II Word List I and II Family Pictures I and II Visual reproduction EXIT

17. Neuropsychological Assessment Presented with slightly stooped body posture, shuffling gait, expressionless face, monotonous voice Co-operative with assessment, high level of motivation, recognized errors but unable to correct them, became distressed FrSBe - family rating done by daughter reflected significant increase in apathy and executive dysfunction, relative to her pre-morbid functioning; self rating form - managed to do half of it (discontinued due to distress), part that has been done was congruent with family rating

18. Neuropsychological Assessment SCIP � moderately severe impairment WAIS III Picture Completion � (SS 6), difficulty with naming, concentrating on peripheral details, missing context, busy describing picture without identifying missing detail, tangential, frequently losing a goal, talking about differences, failing on items involving sequencing Vocabulary � (SS-7) � difficulty in formulating concepts, repetitive use of words (difficulty in changing cognitive set) e.g. designate � �to give instruction to somebody else�, reluctant ��instruction given to somebody but they are not keen to do it�, yesterday � day of the week � 7 days in a week, when we go to be to sleep we wake up the next day, today is finished and then the next day is yesterday.� Digit Symbol coding (SS � 3), made one error copied symbol of the next number instead of the required, recognized the error but unable to correct it

19. Neuropsychological Assessment Similarities (SS � 6) Block Design (SS 3) � Digit Span (SS 7) digits forward 5, backwards 3, Information � (SS 6) Comprehension (SS 7), answers lacking reachness, never given more than one aspect Memory testing WMS III impaired on all of the tests of memory , no evidence of learning, Proactive onterference EXIT -25/30 reflected difficulty with planning

20. Outcome/ overview Both patients ended going to a resthome, prior to this admission both of them lived at home Confirmation that mixed pictures are more common than clear-cut to fit to diagnostic criteria Raises the question of how useful is neuroimaging in diagnosis or maybe what is a critical mass of atrophy or critical change in brain that causes significant changes in behaviour and functioning