Early onset alzheimer’s dementia
•Download as PPTX, PDF•
0 likes•65 views
This document discusses challenges in diagnosing and treating young onset dementia. It defines young onset dementia as occurring before age 65. Two case studies are presented, one with a 53-year-old woman experiencing language difficulties, and another with a 34-year-old woman initially diagnosed with depression but later found to have Alzheimer's disease. Diagnostic challenges include differentiating dementia from other conditions, identifying non-amnestic variants that present atypically, and determining genetic causes. Early onset dementia is often misdiagnosed due to its varied presentations.
Recommended
More Related Content
What's hot
What's hot (19)
Similar to Early onset alzheimer’s dementia
Similar to Early onset alzheimer’s dementia (20)
More from Mohamed Mahdy
Recently uploaded
Recently uploaded (20)
Early onset alzheimer’s dementia
- 1. Dr. Mohammed Mahdy, MD EARLY onset ALZHEIMER’S dementia: challenges in diagnosis and treatment
- 3. DEFINITIONS YOD Young-onset dementia (YOD) refers to patients diagnosed with dementia before 65 years of age. YOD is a relatively common, but frequently misdiagnosed, condition.
- 4. DEFINITIONS YOD Young-onset dementia (YOD) refers to patients diagnosed with dementia before 65 years of age. YOD is a relatively common, but frequently misdiagnosed, condition. PRESENILE? EARLY ONSET?
- 5. DEFINITIONS YOD Young-onset dementia (YOD) refers to patients diagnosed with dementia before 65 years of age. YOD is a relatively common, but frequently misdiagnosed, condition. PRESENILE? Before 45Y EARLY ONSET?
- 6. DEFINITIONS YOD Young-onset dementia (YOD) refers to patients diagnosed with dementia before 65 years of age. YOD is a relatively common, but frequently misdiagnosed, condition. PRESENILE? Before 45Y EARLY ONSET? ● Early-onset Alzheimer disease, which makes up about 5% to 6% of all cases of Alzheimer disease, is distinct from late-onset Alzheimer disease in a number of clinical, genetic, neurobiological, and management features.
- 7. CASE 53y F 2-year history of a progressive decline in her ability to find words and pronounce them correctly. She also could not repeat or understand sentences when they were too long. Investigatio ns neuropsychological testing that showed normal intellectual abilities except for a decline in verbal fluency, with more minor changes in auditory attention and visual memory. unremarkable MRI of the brain, normal routine laboratory results,
- 8. 1st challenge ? Is it dementia? Dementia is a syndrome in which there is deterioration in memory, thinking, behavior and the ability to perform everyday
- 9. 1st challenge ? Is it dementia? Dementia is a syndrome in which there is deterioration in memory, thinking, behavior and the ability to perform everyday ACTU AL G.K. Gouras, in Reference Module in Biomedical Sciences, 2014 Dementia is a chronic decline in cognitive function that causes impairment relative to a person's previous level of social and occupational functioning.
- 11. Episodic Memory ◆ Forgetting recent eventsa ◆ Misplacing personal itemsa ◆ Asking repetitive questionsa ◆ Missing appointmentsa ◆ Paying bills latea ◆ Poor long term/ autobiographical memory
- 12. Episodic Memory ◆ Forgetting recent eventsa ◆ Misplacing personal itemsa ◆ Asking repetitive questionsa ◆ Missing appointmentsa ◆ Paying bills latea ◆ Poor long term/ autobiographical memory Visuospatial ◆ Navigational problems/getting losta ◆ Difficulty locating items in plain sight ◆ Problems visually recognizing faces or objects
- 17. Episodic Memory ◆ Forgetting recent eventsa ◆ Misplacing personal itemsa ◆ Asking repetitive questionsa ◆ Missing appointmentsa ◆ Paying bills latea ◆ Poor long term/ autobiographical memory Visuospatial ◆ Navigational problems/getting losta ◆ Difficulty locating items in plain sight ◆ Problems visually recognizing faces or objects Language ◆ Difficulty retrieving words or namesa ◆ Problems comprehending words or sentences ◆ Effortful or nonfluent speech ◆ Grammar errors or omissions ◆ Spelling errors ◆ Problems reading and writing
- 18. Episodic Memory ◆ Forgetting recent eventsa ◆ Misplacing personal itemsa ◆ Asking repetitive questionsa ◆ Missing appointmentsa ◆ Paying bills latea ◆ Poor long term/ autobiographical memory Visuospatial ◆ Navigational problems/getting losta ◆ Difficulty locating items in plain sight ◆ Problems visually recognizing faces or objects Language ◆ Difficulty retrieving words or namesa ◆ Problems comprehending words or sentences ◆ Effortful or nonfluent speech ◆ Grammar errors or omissions ◆ Spelling errors ◆ Problems reading and writing Executive Functions ◆ Problems organizing, multitasking, or maintaining focusa ◆ Distractibilitya ◆ Difficulty reasoning, problem solving, or making decisionsa ◆ Poor judgment
- 19. Other Cognitive ◆ Problems with calculationsa ◆ Difficulty using devices/technologya ◆ Disorientation to time and plac
- 20. Other Cognitive ◆ Problems with calculationsa ◆ Difficulty using devices/technologya ◆ Disorientation to time and plac Sleep ◆ Insomniaa ◆ Loud snoring/gasping for air ◆ Dream enactment behavior
- 21. Other Cognitive ◆ Problems with calculationsa ◆ Difficulty using devices/technologya ◆ Disorientation to time and plac Sleep ◆ Insomniaa ◆ Loud snoring/gasping for air ◆ Dream enactment behavior General and Autonomic ◆ Weight lossa or gain ◆ Changes in eating behavior and dietary preferences ◆ Positional dizziness ◆ Bladder or bowel incontinence ◆ Sexual dysfunction
- 22. Other Cognitive ◆ Problems with calculationsa ◆ Difficulty using devices/technologya ◆ Disorientation to time and plac Psychiatric/Behavioral ◆ Depressiona ◆ Apathya ◆ Anxietya ◆ Irritabilitya ◆ Agitation ◆ Poor impulse control, lability ◆ Delusions ◆ Hallucinations or misperceptions ◆ Changes in personality ◆ New hobbies or interests ◆ Obsessive or compulsive behaviors ◆ Loss of empathy ◆ Disinhibition ◆ Poor hygiene Sleep ◆ Insomniaa ◆ Loud snoring/gasping for air ◆ Dream enactment behavior General and Autonomic ◆ Weight lossa or gain ◆ Changes in eating behavior and dietary preferences ◆ Positional dizziness ◆ Bladder or bowel incontinence ◆ Sexual dysfunction
- 23. CASE 53y F 2-year history of a progressive decline in her ability to find words and pronounce them correctly. She also could not repeat or understand sentences when they were too long. Investigatio ns neuropsychological testing that showed normal intellectual abilities except for a decline in verbal fluency, with more minor changes in auditory attention and visual memory. unremarkable MRI of the brain, normal routine laboratory results,
- 24. CASE CONT. EXAM List key cost goals, expenditure limits On examination, she had a Mini-Mental State Examination(MMSE) score of 17/30. She could not do the serial reversals and could not come up with the word watch. Memory examination was intact on delayed recall; however, her language examination was quite abnormal. She showed word-finding pauses, hesitations, and frequent phonemic paraphasic errors she had prominent difficulty with repetition, quickly breaking down if a sentence was more than a few words
- 26. Case 2# 34-year-old Jordanian woman who was referred to mainstream mental health services because of irritability, agitation, loss of appetite, withdrawal from family activities and sleeping difficulties. she was initially diagnosed with major depressive disorder but subsequently showed very poor response to antidepressant therapy. Her presentation gradually and dramatically progressed into full blown dementia within couple of years. Brain MrI showed atrophic cortical changes and subcortical white matter alterations consistent with alzheimer’s dementia. Brain pet scan revealed reduction in cerebral glucose metabolism in temporoparietal areas bilaterally most consistent with alzheimer’s dementia. there was a strong family history
- 28. TIME COURSE
- 29. DD
- 30. DD
- 31. DD
- 32. DD
- 33. DD
- 34. DD
- 35. DD
- 36. ● Logopenic variant primary progressive aphasia, the most common nonamnestic phenotypic variant of early- onset AD, presents with a progressive decline in language with relatively spared memory and cognition due to focal AD neuropathology in temporoparietal language areas in the left hemisphere, especially the superior/ midtemporal gyrus, angular gyrus, and midfrontal cortex.
- 37. ● Logopenic variant primary progressive aphasia, the most common nonamnestic phenotypic variant of early- onset AD, presents with a progressive decline in language with relatively spared memory and cognition due to focal AD neuropathology in temporoparietal language areas in the left hemisphere, especially the superior/ midtemporal gyrus, angular gyrus, and midfrontal cortex. ● Posterior cortical atrophy, the second most common early-onset Alzheimer disease variant, presents with progressive and disproportionate loss of visuospatial or visuoperceptual functions, usually due to Alzheimer neurodegeneration of posterior visual cortical regions.
- 38. ● Logopenic variant primary progressive aphasia, the most common nonamnestic phenotypic variant of early- onset AD, presents with a progressive decline in language with relatively spared memory and cognition due to focal AD neuropathology in temporoparietal language areas in the left hemisphere, especially the superior/ midtemporal gyrus, angular gyrus, and midfrontal cortex. ● Posterior cortical atrophy, the second most common early-onset Alzheimer disease variant, presents with progressive and disproportionate loss of visuospatial or visuoperceptual functions, usually due to Alzheimer neurodegeneration of posterior visual cortical regions. ● The frontal variant of Alzheimer disease, now known as behavioral/ dysexecutive Alzheimer disease, presents with features suggestive of frontotemporal lobar degeneration but most commonly with apathy or abulia.
- 41. CHALLENGES
- 42. 1st Diagnosis
- 43. ◆ Large percentage of nonamnestic phenotypic variants (logopenic variant primary progressive aphasia, posterior cortical atrophy, behavioral/dysexecutive, acalculia, corticobasal syndrome)
- 44. ◆ Large percentage of nonamnestic phenotypic variants (logopenic variant primary progressive aphasia, posterior cortical atrophy, behavioral/dysexecutive, acalculia, corticobasal syndrome) ◆ Genetic predisposition: About 1 in 10 patients has an autosomal dominant familial Alzheimer disease (PSEN1, PSEN2, APP), and there is a high polygenic risk score of susceptibility genes
- 45. ◆ Large percentage of nonamnestic phenotypic variants (logopenic variant primary progressive aphasia, posterior cortical atrophy, behavioral/dysexecutive, acalculia, corticobasal syndrome) ◆ Genetic predisposition: About 1 in 10 patients has an autosomal dominant familial Alzheimer disease (PSEN1, PSEN2, APP), and there is a high polygenic risk score of susceptibility genes ◆ More aggressive course with high rate of mortality
- 46. ◆ Large percentage of nonamnestic phenotypic variants (logopenic variant primary progressive aphasia, posterior cortical atrophy, behavioral/dysexecutive, acalculia, corticobasal syndrome) ◆ Genetic predisposition: About 1 in 10 patients has an autosomal dominant familial Alzheimer disease (PSEN1, PSEN2, APP), and there is a high polygenic risk score of susceptibility genes ◆ More aggressive course with high rate of mortality ◆ Delay in diagnosis of about 1.6 years
- 47. ◆ Higher prevalence of traumatic brain injury (which lowers age of onset) and lower vascular risk factors
- 48. ◆ Higher prevalence of traumatic brain injury (which lowers age of onset) and lower vascular risk factors ◆ Overall less semantic memory impairment and greater attention, executive, praxis, and visuospatial difficulties
- 49. ◆ Higher prevalence of traumatic brain injury (which lowers age of onset) and lower vascular risk factors ◆ Overall less semantic memory impairment and greater attention, executive, praxis, and visuospatial difficulties ◆ Greater psychosocial problems (unexpected midlife “out-of- step” loss; continued work, financial, family responsibilities; retained insight with depression, anxiety, suicide risk)
- 50. ◆ Higher prevalence of traumatic brain injury (which lowers age of onset) and lower vascular risk factors ◆ Overall less semantic memory impairment and greater attention, executive, praxis, and visuospatial difficulties ◆ Greater psychosocial problems (unexpected midlife “out-of- step” loss; continued work, financial, family responsibilities; retained insight with depression, anxiety, suicide risk) ◆ Hippocampal sparing and less mesial temporal lobe disease ◆ Greater posterior (parietal, temporoparietal junction) neocortical atrophy and hypometabolism versus temporal atrophy and hypometabolism
- 51. ◆ Higher burden of tau/neurofibrillary tangles per gray matter atrophy and stage of dementia, especially in focal phenotypic areas (reflected in tau imaging)
- 52. ◆ Higher burden of tau/neurofibrillary tangles per gray matter atrophy and stage of dementia, especially in focal phenotypic areas (reflected in tau imaging) ◆ Greater involvement of white matter tracts in posterior association areas and frontoparietal networks and greater involvement of non–default mode neural networks rather than the default mode network
- 53. 2nd DD
- 58. 3rd Treatment
- 59. How to treat? How did that work? Right set of people? Strategies for pharmacologic management of YOD are similar to those for late-onset dementia; currently, no YOD- specific pharmacologic therapies are available. In general, pharmacologic management strategies include the use of cholinesterase inhibitors (ChEIs) and the N- methyl-D-aspartate (NMDA) antagonist, memantine (ie, antidementia drugs), and other
- 60. The challenge
- 62. How to treat? How did that work? Right set of people? Strategies for pharmacologic management of YOD are similar to those for late-onset dementia; currently, no YOD- specific pharmacologic therapies are available. In general, pharmacologic management strategies include the use of cholinesterase inhibitors (ChEIs) and the N- methyl-D-aspartate (NMDA) antagonist, memantine (ie, antidementia drugs), and other Donanemab Aducanumab
- 67. Migraine
- 71. Dementia
- 74. Key Lessons
- 75. • YOD is a challenge in diagnosis (don’t wait for memory defect)
- 76. • YOD is a challenge in diagnosis (don’t wait for memory defect) • YOD is a challenge in DD (Don’t forget PNLE,NPNLE,Vascular)
- 77. • YOD is a challenge in diagnosis (don’t wait for memory defect) • YOD is a challenge in DD (Don’t forget PNLE,NPNLE,Vascular) • YOD is a challenge in ttt (don’t stick hardly to rules, new hope in the horizon)