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A Case on DEMENTIA. Neurology MIRANDA – MOLINA – MONZON – MORALES – MUSNI – NALLAS - NAVAL Batch 2011 - Section C. History. A 63 y/o woman was brought by her husband for consult because of increasing forgetfulness.
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A Case onDEMENTIA Neurology MIRANDA – MOLINA – MONZON – MORALES – MUSNI – NALLAS - NAVAL Batch 2011 - Section C
History A 63 y/o woman was brought by her husband for consult because of increasing forgetfulness. The husband reports that his wife had completed a degree in BS Education. She has been teaching for the past 25 yrs. Confidentially, he reports that she has increasing difficulty remembering her class schedules and examinations as well as conversations with coworkers over the past year. He likewise noted reduced interest and withdrawal from many long-standing social activities. Recently, she left food cooking on the stove, which resulted in a small kitchen fire.
History The patient has no significant current medical problems and takes no medications. The patient's older brother has recently been diagnosed with the same illness.There were no significant PE findings. On mental status testing, the patient was noted to be disoriented to time and person. She had difficulty with calculation and had impaired short-term verbal memory. Visuospatial abilities, however, were intact. Cranial CT scan done revealed normal findings.
Salient FeaturesPertinent Positive • 63 year old, female • CC: Increasing forgetfulness over the past year • Difficulty remembering class schedules and exams and conversations with co-workers • Left food cooking on the stove small kitchen fire • Impaired short-term verbal memory • Reduced interest ; withdrawal from social activities • Disoriented to person and time • Difficulty with calculation • Brother: diagnosed with same illness
Salient FeaturesPertinent Negative • No significant medical problems • No medications taken • No significant PE findings • Visuospatial abilities intact • Normal CT
Salient FeaturesPertinent Positive • 63 year old, female • CC: Increasing forgetfulness over the past year • Impaired short-term verbal memory • Reduced interest; withdrawal from social activities • Disoriented to person and time • Difficulty with calculation • Brother: diagnosed with same illness Memory Impairment
Memory Impairment Memory Impairment Reference
Salient FeaturesPertinent Positive • 63 year old, female • CC: Increasing forgetfulness over the past year • Impaired short-term verbal memory • Reduced interest; withdrawal from social activities • Disoriented to person and time • Difficulty with calculation • Brother: diagnosed with same illness Memory Impairment Pseudodementiais a depression-related cognitive dysfunction Reference
Dementia • Syndrome of cognitive decline with variable non-cognitive features of behavioral and psychiatric symptoms and disturbance in activities of daily living Reference
Criteria for Dementia DSM –IV criteria Patient Increasing forgetfulness over the past year Difficulty remembering class schedules exams conversations with co-workers Attention: Left food cooking on the stove small kitchen fire Reduced interest ; withdrawal from social activities Disoriented to person and time Difficulty with calculation Impaired short-term verbal memory • Multiple Cognitive Deficits • Memory Impairment • One or more • Aphasia • Apraxia • Agnosia • Executive • Impaired Social/Occupational function • Gradual and progressive course
Classification Disease in which Dementia is: • Associated with clinical and laboratory signs of other medical diseases • Associated with other neurological signs but not with other obvious medical disorders • Invariable associated with other neurologic signs • Often associated with other neurologic signs • Usually the only evidence of neurologic or medical diseases • Pertinent Negatives • No significant medical problems • No medications taken • No significant PE findings • Normal CT Principles of Neurology , 8th Edition
Dementia Dementia is usually the only evidence of neurologic or medical disease • Alzheimers Disease • Diffuse Lewy Body Dementia • Pick Disease • Frontotemporal and frontal lobe dementias Reference
Dementia • Diffuse Cerebral Atrophy • Alzheimers Disease • Diffuse Lewy Body Dementia • Circumscribed Cerebral Atrophy • Pick Disease • Frontotemporal and frontal lobe dementias Reference
Dementia: Usually the only evidence of neurologic or medical diseases Reference
Alzheimer’s DiseaseDSM IV Criteria A. The development of multiple cognitive deficits manifested by both: -1.Memory impairment (impaired ability to learn new information or to recall previously learned information) -2.One or more of the following cognitive disturbances: (a) aphasia (language disturbance) (b) apraxia (impaired ability to carry out motor activities depite intact motor function) (c) agnosia (failure to recognize or identify objects despite intact sensory function) (d) disturbance in executive functioning (i.e., planning, organizing, sequencing, abstracting) B. The cognitive deficits in criteria A1 and A2 each cause significant impairment in social or occupational functioning and represent a significant decline from a previous level of functioning. C. The course is characterized by gradual onset and continuing cognitive decline. D. The cognitive deficits in Criteria A1 and A2 are not due to any of the following: (a) other central nervous system conditions that cause progressive deficits in memory and cognition (e.g., cerebrovascular disease, Parkinson's disease, Huntington's disease, subdural hematoma, normal-pressure hydrocephalus, brain tumor) (b) systemic conditions that are known to cause dementia (e.g., hypothyroidism, vitamin B or folic acid deficiency, niacin deficiency, hypercalcemia, neurosyphilis, HIV infection) (d) substance-induced conditions E. The deficits do not occur exclusively during the course of a delirium. F. The disturbance is not better accounted for by an Axis I disorder Reference
Alzheimer’s DiseaseDSM IV Criteria A. The development of multiple cognitive deficits manifested by both: -1.Memory impairment (impaired ability to learn new information or to recall previously learned information) -2.One or more of the following cognitive disturbances: (a) aphasia (language disturbance) (b) apraxia (impaired ability to carry out motor activities despite intact motor function) (c) agnosia (failure to recognize or identify objects despite intact sensory function) (d) disturbance in executive functioning (i.e., planning, organizing, sequencing, abstracting) B. The cognitive deficits in criteria A1 and A2 eachcause significant impairment in social or occupational functioning and represent a significant decline from a previous level of functioning. C. The course is characterized by gradual onset and continuing cognitive decline. D. The cognitive deficits in Criteria A1 and A2 are not due to any of the following: (a) other central nervous system conditions that cause progressive deficits in memory and cognition (e.g., cerebrovascular disease, Parkinson's disease, Huntington's disease, subdural hematoma, normal-pressure hydrocephalus, brain tumor) (b) systemic conditions that are known to cause dementia (e.g., hypothyroidism, vitamin B or folic acid deficiency, niacin deficiency, hypercalcemia, neurosyphilis, HIV infection) (d) substance-induced conditions E. The deficits do not occur exclusively during the course of a delirium. F. The disturbance is not better accounted for by an Axis I disorder Reference
Alzheimer’s Disease • Most common cause of dementia • Incidence increases with age • 60 years and above • 3x higher in women • (+) Family History • Chromosome 21 – amyloid gene – senile plaques • Chromosome 19 – ApoE4 gene – inherited predisposition • Chromosome 1, 14 – Presenilins 1 and 2 Reference
Alzheimer’s Disease Risk Factors • Old age • Family history • Low education • Head trauma • High cholesterol • Hypothyroidism • Exposure to metals Reference
Alzheimer’s Disease Risk Factors • Old age – 63 yrs old • Family history – older brother • Low education • Head trauma • High cholesterol • Hypothyroidism • Exposure to metals Reference
Alzheimer’s Disease Clinical Features • Gradual development of forgetfulness • Cognitive Dysfunctions • Language: expression, comprehension, reading, writing • Decline in arithmetic skills (acalculia/dyscalculia) • Visuospatial orientation • 4 A’s: amnesia, aphasia, apraxia, agnosia Reference
Alzheimer’s Disease Clinical Features • Gradual development of forgetfulness • Cognitive Dysfunctions • Language: expression, comprehension, reading, writing • Decline in arithmetic skills (acalculia/dyscalculia) • Visuospatial orientation • 4 A’s: amnesia, aphasia, apraxia, agnosia Reference
Alzheimer’s Disease Clinical Features • Executive Dysfunction • Planning • Organizing • Sequencing • Abstract thinking • Behavioral and personality change • Decline in ADL Reference
Alzheimer’s Disease Clinical Features • Executive Dysfunction • Planning • Organizing • Sequencing • Abstract thinking • Behavioral and personality change • Withdrawal from social activities • Decline in ADL • Disturbance in the household and workplace Reference
Alzheimer’s Disease Pathophysiology • Generalized brain atrophy • Loss of neurons • Astrocytic proliferation - inflammation • Microscopic changes - Neurofibrillary tangles • Histological marker - Amyloid deposition • Histological marker - Granulovacuolar degeneration Reference
Alzheimer’s Disease • Diagnostic Procedures • Cranial CT or MRI scan - Mild AD:normal or MTL atrophy - Advanced AD • Generalized atrophy • EEG - Diffuse slowing (theta/delta range) in late disease • CSF analysis - Normal, slight increase in total protein Reference
Alzheimer’s Disease Diagnostic Procedures • Neuropsychological Tests - Poor memory, verbal skills in early to moderate stages • Biologic Markers - CSF tau and β amyloid - Inflammatory markers • Isopostane (serum & CSF) Reference
Diagnostic Procedures • Mini-Mental State Examination • Blood tests • Cranial CT scan or MRI • Single-photon emission CT (SPECT) • EEG • CSF analysis Reference
Mini-Mental State Examination • assesses cognitive abilities such as orientation to time and place, use of language, memory, attention, and abilities to carry out various tasks and follow instructions Reference
Blood tests • check for infections or conditions such as vitamin deficiency, anemia, medication levels, disorders of the thyroid, kidneys or liver Reference
Cranial CT scan or MRI • reveals reduction in the size of the brain (atrophy), widened indentations in the tissues, and enlargement of the cerebral ventricles Reference
Single-photon emission CT (SPECT) • imaging detects blood flow in the brain • used in some medical centers to distinguish Alzheimer’s disease from vascular dementia Reference
EEG • diffuse slowing (theta/delta range) in late disease Reference
CSF analysis • normal, slight increase in total protein • biologic markers: amyloid beta or tau proteins Reference