Early onset dementia in Down syndrome -Dental management considerations ASSCID Walkabout Conference 2017 Brisbane, Australia

Transcription

1 Early onset dementia in Down syndrome -Dental management considerations ASSCID Walkabout Conference 2017 Brisbane, Australia Dr Zanab Malik, BDS Resident- Department of Oral Medicine, Oral Pathology and Special Needs Dentistry Westmead Centre for Oral Health, Westmead, Sydney Australia

2 Introducing Ms K 48 year old female C/O: Possible oral pathology leading to challenging behaviour Medical history: Allergies+ Vancomycin (rash) CVS CNS+ Down syndrome, Moderate intellectual disability Challenging behaviours (Zyprexa) Poorly controlled epilepsy (Lamictal, Epilim) Endocrine+ GIT+ Hypothyroidism (Oroxine), Clinical obesity (BMI >30; Normal 18.5 to 25). Unable to assess for Diabetes GORD (Losec), GUT Renal - Hepatic - Infectious diseases Immune - Skin Cancer Musculoskeletal Recent hospitalisations + Prolonged in 2013, for fractured ankle, subsequent increased challenging behaviour and personality change

3 Patient Case Social History: Lives with her mother Mother is consenting guardian for medical and dental treatment 5 day/week day program and regular respite Assistance with ADLs Independent for mobility, continence and feeding Oral Care: Daily, 1000ppm F Independent oral hygiene with prompting

4 Examination Challenging /aggressive behaviour at appointment kicking and hitting staff Extra-oral examination: nil frank facial asymmetry/swelling Limited visual examination of anterior intra-oral tissues: mature plaque deposits with gingivitis Radiographic examination not possible

5 DDx: Oral and Systemic Underlying oral pathology History not consistent with oral pathology absence of pain history or hallmark signs of oral pain: altered sleep or dietary changes Pending GA to confirm absence of oral pathology Ms K showed ONLY behaviour change Avoid diagnostic overshadowing* (*Negative bias impacting our judgement regarding co-occurring disorders especially in patients with intellectual disabilities or mental illness) Underlying systemic pathology Neurocognitive cause of behaviour/personality change *Family history of Alzheimer's disease helped to confirm a presumed working diagnosis of Alzheimer's disease in this case

6 Down syndrome and Alzheimer s disease, the connection Amyloid Precursor Protein (APP) production linked to chromosome 21 Early ageing and early onset of AD associated with Down syndrome Characteristic plaques and tangles seen earlier in Down syndrome Content/WWW/CMS/images/New_Pic.jpg eature.jpg

7 Prevalence In individuals with DS over age 35, 25% display symptoms associated with Alzheimer s disease Incidence of Alzheimer s disease is estimated at 3-5 times that of the general population 50% of people with DS will develop Alzheimer s by age 60 Cumulative Incidence of Dementia/AD in adults with Down syndrome (Red), the overall population (Yellow) and other ID (Green) Silverman, 2010

8 Presentation of Alzheimer s disease what is prominent in Down syndrome? Personality Changes: Reduced empathy, apathy Social withdrawal Emotional instability Restlessness or sleep disturbance Change in coordination and walking Seizures that begin in adulthood

9 Diagnosis of Alzheimer's disease in Down syndrome Eliminate other causes of decline in functioning Standard tests; Mini-Mental Status Examination (MMSE), used in the general population are often not appropriate Early assessments are crucial for comparison Specific diagnostic tests include: Dementia Scale for Down syndrome (DSDS) Test for Severe Impairment (TSI) Down Syndrome Mental State Exam (DSMSE) Dementia Questionnaire for People with Learning Disabilities (DLD)

10 Ms K Dental Management options No treatment in the absence of symptoms Premedication prior to future appointment to attempt a more comprehensive examination IV sedation unsuitable General anaesthetic for comprehensive assessment Examination Scale and clean X-rays Fluoride treatment Restoration of any teeth with dental caries Extraction of any teeth with hopeless prognosis Value add: Blood tests, assess for Diabetes, medical imaging

11 Risks vs Benefits of GA for dental treatment Consideration of GA risks: Post-operative apnoea given clinical obesity Post-operative cognitive dysfunction Post-operative challenging behaviour plan for close monitoring at home with increased support services vs overnight ward admission Post operative pain management

12 Management Goals Ettinger R, 2006, Rational Dental Care Part 1. Has the concept changed in 20 years?, Journal of Canadian Dental Association, 72 (5);

13 Conclusion Management of challenging behaviour in patients requires multidisciplinary team approach In the setting of Down syndrome, behavioural changes in patients over 35 years should prompt investigation of possible underlying neurodegenerative disease Eliminate possible organic oral or systemic pathology as a cause of pain or behaviour change For the dental practitioner, patients with Down syndrome and early-onset dementia present a unique challenge in synthesizing principles of dental management in the setting of developmental disability and neurodegenerative disease.

14 Acknowledgements- Dr Avanti Karve, Staff Specialist Special Needs Dentistry, Westmead Centre for Oral Health A/Prof Mark Schifter, Head of Department, Staff Specialist Oral Medicine, Westmead Centre for Oral Health Ms Natalie Jaksic, OHT, Westmead Centre for Oral Health Dr Linda Gu, Senior Registrar, Special Needs Dentistry, Westmead Centre for Oral Health Dr Helen Sommerville, Developmental paediatrician and disability physician

15 References Alzheimers Australia, Down Syndrome and Alzheimer s disease, -dementia/types-of-dementia/downsyndrome-and-alzheimers-disease, (accessed 13 th August 2017). Alzheimer Society, 2016, Down syndrome and Alzheimer s disease, Alzheimer Society of Canada, Nov issue, pg 1-8 Lott IT & Head E, 2001, Down syndrome and Alzheimer s disease: a link between development and aging, Mental Retardation and Developmental Disabilities Research Reviews, 7(3): McCarron et al, 2017, A prospective 20-year longitudinal follow-up of dementia in persons with Down syndrome, Journal of Intellectual Disability Research, Silverman W, 2010, Aging and end of life webinar series, Dementia Among Adults with Down syndrome: Individual differences in risk and progression, John Hopkins University School of Medicine. Stanton LR & Coetzee RH, 2004, Down s syndrome and dementia, Advances in Psychiatric Treatment, vol 10, pp Torr J et al, Down syndrome and Alzheimer s disease, Alzheimer s Australia, Down Syndrome Australia and the Centre for Developmental Disability Health Victoria (CDDHV).