Mixed Dementia
Vascular Cognitive Impairment¶
- Features Early gait impairment and personality/mood changes Slowed processing speed
- Imaging No hippocampal atropy and presence of confluent white matter hyperintensities on MRI of the brain
Vascular Dementia (VaD)¶
- 10% of all dementia, >60 y/o
- Higher risk in men and more likely to cause motor symptoms
- Primarily Executive/Cognitive Dysfunction
- Symptoms Stepwise (not gradual) decline w/focal neurological deficits Early Executive Dysfunction o 2 Main Types: A) Multi-infarct Dementia • Abrupt onset, stepwise deterioration • Typically have prominent motor, reflex, visual, and gait abnormalities • Do not involve problems naming objects (Alzheimer’s) • More prominent fluctuation of cognition B) Binswanger Disease (Diffuse White Matter) • Associated with longstanding hypertension and/or atherosclerosis, diabetes • Diffuse subcortical white matter damage and subacute insidious course • Lacunar infarcts can be seen on MRI • Early: confusion, impaired memory, perception, executive functions • Late: difficulty in judgement and orientation • Apathy, anxiety, psychosis, euphoria, depression, aggression • Urinary incontinence and dysarthria with or without dysphagia, emotional lability o Cerebral infarction and/or deep white matter changes on imaging Multiple small cerebral infarctions from cardiovascular disorders No ventriculomegaly/generalized atrophy in early disease
- Treatment
- Antihypertensives, anticoagulants
Creutzfeldt-Jacob Disease (CJD)¶
o Prion Disease sCJD (Sporadic): 95% of all, 55-65 y/o fCJD (Familial): 5% of all iCJD (iatrogenic) and vCJD (Variant), Younger patients • vCJD caused by mad cow disease • iCJD caused by infected human tissues or hormones o Symptoms Rapid progression of psychiatric symptoms with anxiety, fatigue, ataxia followed by dementia within weeks Akinetic Mutism Pyramidal/extrapyramidal dysfunction Cerebellar dysfunction, hiccups Hypokinesia, Startle Myoclonus late in disease course Circadian rhythm interruption Cerebellar ataxia, decreased tendon reflexes o Diagnosis: Real Time Quaking-Inducted Conversion Assay of CSF • Most sensitive and specific for prion proteins o Uses recombinant prion protein and thioflavin T, fluorescent dye • Elevated CSF of 14-3-3 regulatory proteins, total tau proteins o Not sensitive or specific enough Confirmatory: Brain Biopsy Generalized slowing with periodic, bi/tri phasic sharp wave complexes on EEG, “Triphasic Bursts” T1/T2 MRI: abnormal diffusion in thalmi, basal ganglia, or cortical ribbon • Increased intensity in the diffusion-weighted sequence in the BG and various cortical regions o Prognosis: fatal in <1 year in >90% of patients o Treatment None • Alcoholic Dementia o Ethanol Induced Dementia 5-6 drinks a day needed over a lifetime • Hypoxic Encephalopathy • AIDS Dementia Complex o 2% of all dementia, mcc in young patients Affects half of all AIDs patients not on ART o Cortical and subcortical atrophy and ventricular enlargement Subacute or chronic cognitive defects, depression, impaired psychomotor ability Irreversible dementia, subcortical symptoms, Basal ganglia or nigrostriatal dysfunction Slower movement, difficult smooth limb actions Related to the degree and duration of immunosuppression o HIV-Associated Neurocognitive Disorders (HAND) Dementia due to subcortical atrophy, inflammation and demyelination • Basal ganglia or nigrostriatal dysfunction • Can be prevented in HIV but not AIDS RF: Long standing HIV, Age ≥50, CD4 < 200 Severity (increasing as you go down): • Asymptomatic neurocognitive impairment • Mild neurocognitive disorder • HIV-associated Dementia/Encephalopathy Symptoms • Impaired memory and attention/concentration, anhedonia o Personality and behavior changes • Decreased alertness, confusion, blunted affect, slowed movement, impaired concentration, generalized atrophy • Movement disorders later (tremor and gait) o Slower movements with substantial cognitive impairment late Prognosis: Quicker progression, months, life expectancy <1 year Diagnosis: Clinical, neuropsychological testing • MRI: diffuse increase in white matter intensity o Diffuse atrophy later Treatment • ART with high CNS uptake • Cortico-Basal Syndrome o Asymmetric posterior frontoparietal degeneration
Traumatic Encephalopathy Syndrome (TES)¶
- Progressive neurodegenerative syndrome with repetitive head trauma
- Emotional dysregulation, behavior change, motor disturbance w/parkinsonian features
- Memory features largely lacking
- Generalized cerebral atrophy, MRI may be normal