Dementia - DEMENTIA DEMENTIA Kefah Al-Hayek, MD JUST...

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Unformatted text preview: DEMENTIA DEMENTIA Kefah Al-Hayek, MD JUST Definition Definition Acquired generalized and often progressive Acquired impairment of cognitive function that affects the content, but not the level, of consciousness. content but level DSM-IV Criteria for Dementia: DSM-IV 1. The gradual onset and continuing decline of The cognitive function from a previously higher level, resulting in impairment in social or occupational function function 2. Impairment of recent memory (the inability to Impairment learn new information), and at least one of the following: following: a) Language (word-finding difficulties) b) Disturbances of praxis (inability to execute Disturbances skilled motor activities in the absence of weakness) weakness) DSM-IV Criteria for Dementia: DSM-IV (cont.) c) Disturbances of visual processing (visual agnosia Disturbances and constructional disturbances) and d) Disturbances of executive function (including Disturbances abstract reasoning and concentration) abstract 1. The cognitive deficits are not due to other The psychiatric disease, neurologic diseases, or systemic diseases, and the deficits do not exclusively occur in the setting of delirium exclusively Mild Cognitive Impairment Mild MMSE 24-28 Problem with recollection not meet dementia criteria not Does Does Differential Diagnosis: Differential 1. 2. 3. 4. 5. 5. 6. 7. Alzheimer Disease (pure ~40%, + mixed~70%) Vascular Disease, MID (5-20%) Drugs, Depression, Delirium rugs, epression, Ethanol Medical / Metabolic Systems edical Endocrine (thyroid, diabetes) Neurologic, Nutritional (other primary eurologic, degenerations, etc.) degenerations, 8. Tumor, Toxin, Trauma umor, oxin, 9. Infection, Idiopathic, nfection, 10. Amnesia, Autoimmune, 10. mnesia, utoimmune, Treatable causes Treatable ~ 15% of causes NPH IC mass lesion (tumor, SDH) B12 def. Hypothyroidism Syphilis Depression Estimate MMSE as a function of time MMSE score 30 25 20 15 10 5 0 -10 -8 -6 -4 -2 0 2 4 6 Estimated years into illness AAMI / MCI DEMENTIA 8 10 Evaluation Good history and physical CBC, chem. 18 (with electrolytes and LFTs), TFTs, CBC, B12, RPR/VDRL, ESR, ANA, RF B12, Time course, associated symptoms, PMH, Drugs Family Hx and good mental status examination Family Young pts: Wilson's work-up, porphyria work-up, 24-hour Young urine for heavy metals, HIV, PPD, ACE level, vitamin E CT &/or MRI EEG EKG Evaluation (cont.) Evaluation Urinalysis LP (with cytology, AFB and fungal stains, LP ACE level, MS profile, etc.) ACE Neuropsychological testing if trouble with Neuropsychological diagnosis Arteriogram/Brain biopsy as indicated Arteriogram/Brain Dementia Vs acute confusional state state Alzheimer’s Disease (AD) (AD) Alzheimer’s Disease (AD) Alzheimer’s Alzheimer’s Disease (AD) Alzheimer’s Epidemiology Epidemiology Most common degenerative disease of the brain Most 10% of people over age 65 years have AD 20% of persons > 80 years 20% 30% of > 90 years old 30% Male = Female Male Histopathology: Histopathology: Macroscopic: Atrophy, mostly temporoparietal and frontal Microscopic Loss of neurones and synapses Neurofibrillary tangles (NFTs) amyloid plaques ( Neurotic plaques) Granulovsacular degeneration Chemistry loss of neurotransmitters, especially acetylcholine loss (ACh) (ACh) Tangles Tangles AD: a progressive CNS disorder AD: with a characteristic pathology Brain atrophy Senile plaques Neurofibrillary tangles Katzman, 1986; Cummings and Khachaturian, 1996 Numerous tangles in hippocampus Numerous Amyloid plaque Amyloid Risk factors for AD: Down’s Down’s syndrome Strong risk factors for AD* Strong Age Down’s syndrome Family history Certain genes associated with increased risk Certain but do not cause AD e.g. ApoE4 Genetics Genetics Clinical features of AD Clinical Gradual decline of intellectual function Poor short-term memory Visuospatial disorientation Language/speech problems—aphasia, anomia, and later Language/speech echolalia, mutism Apraxias—dressing, ideomotor Personality changes: indifference, apathy Psychiatric: Hallucination, delusions, behavioural Psychiatric: disturbances disturbances limb rigidity, flexion posture Urinary and faecal incontinence MRI: profound atrophy MRI: Treatment Treatment No cure Anticholinesterases Tacrine, Donepezil, Rivastigmine Alpha tocopherol (vitamin E) Selegiline Selegiline Psychotropic drugs: agitation, delusions Antidepressants Nursing home Dementia with Lewy bodies bodies Dementia with Lewy bodies Up to 20% of dementias Dementia (as previously defined) Lewy bodies diffusely through cortex cortex Markedly fluctuating cognitive impairment Markedly Visual and/or auditory hallucinations Paranoid delusions Falls Extrapyramidal features: rigidity & bradykinesia Extrapyramidal rigidity Neuroleptic sensitivity: extrapyramidal S/Es Vascular dementia Vascular Vascular dementia Vascular Also called multi-infarct dementia (MID) About 15-20% Decline can be linear or step-wise Vascular dementia Vascular Step-wise decline Step-wise - uneven steps, varying plateau uneven VaD -vs.- AD Earlier onset than AD and M > F Sudden onset, stepwise deterioration h/o high bp, strokes Evidence associated arteriosclerosis, eg Evidence coronary artery disease coronary Focal neurological symptoms and signs Focal pathology on brain imaging Fronto-temporal dementia Pick’s disease Pick’s Fronto-temporal dementia Fronto-temporal Pick’s disease Pick’s Macroscopic and brain scan - atrophy only in frontal and temporal areas (until late in disease) disease) Diagnosis easily missed initially Histopathology: Pick cells Pick Pick inclusion bodies No amyloid plaques nor NFT No Rx Fronto-temporal dementia Pick’s disease (cont.) Pick’s Onset 40-60 y.o. (20-80 y. range) Preservation of memory until late Preservation Early symptoms behavioural or psychiatric Early behavioural psychiatric Apathy, irritability Loss of concern Impaired judgement and insight Language affected Creutzfeldt-Jakob disease disease Creutzfeldt-Jakob disease Creutzfeldt-Jakob Transmissible: cornea transplants, intracerebral Transmissible: recording electrode implants, growth hormone recording Rapidly progressive dementia Rapidly Focal involvement of the cerebral cortex, basal ganglia, Focal cerebellum, brainstem, and spinal cord cord Etiologic agent: proteinaceous infectious particle Etiologic (prion) PrPc Cellular isoform Mutation leads to accumulation of PrPSc Familial cases PrPSc Creutzfeldt-Jakob disease (cont.) Creutzfeldt-Jakob Dementia Psychiatric symptoms Myoclonus Extrapyramidal signs Cranial nerve palsies New Variant: bovine spongiform encephalopathy bovine Earlier onset (mean age, about 30 years) More prolonged course (median duration over 1 year) Prominent early psychiatric abnormalities, including Prominent depression and personality changes. depression Creutzfeldt-Jakob disease (cont.) Creutzfeldt-Jakob Investigation: EEG: periodic sharp waves and spikes Sc Detection of PrPSc in brain tissue in Prognosis: Invariably fatal Death w/n 1 yr NORMAL-PRESSURE HYDROCEPHALUS HYDROCEPHALUS NORMAL-PRESSURE HYDROCEPHALUS HYDROCEPHALUS Sometimes called communicating (lateral, 3rd, 4th ventricles remain in communication) or nonobstructive hydrocephalus Triad Dementia Gait apraxia Incontinence Incontinence Idiopathic or secondary (meningitis, SAH) Memory defects, but rarely aphasia and agnosia NORMAL-PRESSURE HYDROCEPHALUS (cont.) HYDROCEPHALUS Weeks-months Gait apraxia early, with weight bearing Pyramidal signs Pyramidal Urinary incontinence Urinary Lp: normal or low opening pressure Remove 30-50 cc; prognostication CT scan or MRI: enlarged lateral ventricles without CT increased prominence of cortical sulci increased nence Cisternography: delayed clearance Rx: CSF shunting; VA,VP,LP THE END THE ...
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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