Alzheimer's Disease | Vascular Dementia (multi-infarct dementia) | Vascular Dementia (lacunar disease) | Binswanger's | Binswanger's | Parkinson's Disease | Dementia with Lewy Bodies | Normal Pressure Hydrocephalus | Hydrocephalus Ex-Vacuo | Corticobasal Degeneration (CBD) | Huntington's | Frontotemporal Lobar Degeneration | Frontotemporal Dementia (FTD) | Pick's | Primary Progressive Aphasia | Semantic Dementia | Progressive Nonfluent Aphasia | B-12 | Thyroid | Creutzfeldt-Jakob | Lyme | Delirium | Shy-Drager syndrome | DLB disease | olivopontocerebellar atrophy (OPCA) | multiple sclerosis | brainstem neoplasm | progressive supranuclear palsy (PSP) | FXTAS | Notes | Source | |||||
Cause | amyloid beta plaques & tau tangles | multiple strokes | multiple strokes | Cerebrovascular lesions deep in the white matter of the brain | Cerebrovascular lesions deep in the white matter of the brain | Lewy bodies | Lewy bodies, amyloid beta plaques | subarachnoid hemorrhage, head trauma, infection, tumor, complications of surgery, injury, unknown (idiopathic0 | Stroke, traumatic injury | Genetic | (inherited) | Pick's bodies (variant of FTD) | (variant of FTD) | a variant of primary progressive aphasia (variant of FTD) | vitamin B-12 deficiency | Genetic, mostly men | |||||||||||||||||||
Physical Characteristics | |||||||||||||||||||||||||||||||||||
Amyloid beta plaques | Cause | Often present | |||||||||||||||||||||||||||||||||
Atrophy | cell loss and shrinkage (atrophy) in certain areas of the brain (cerebral cortex and substantia nigra), asymmetric atrophy | frontal, temporal | asymmetric (worse on left) anterolateral temporal lobe atrophy | left side of the brain is affected most | |||||||||||||||||||||||||||||||
basal ganglia | |||||||||||||||||||||||||||||||||||
disease of medium-sized vessels | Cause | multi-infarct dementia | |||||||||||||||||||||||||||||||||
disease of small vessels | Cause | uncommon variant of small-vessel dementia | uncommon variant of small-vessel dementia | lacunar disease | |||||||||||||||||||||||||||||||
Distended ventricles | distended ventricles without enlarged subarachnoid space | ||||||||||||||||||||||||||||||||||
fronto-parietal regions of the brain's cortex | asymmetric atrophy | ||||||||||||||||||||||||||||||||||
glial inclusions | present, but role unknown, not immunoreactive to ubiquitin | present, role unknown | |||||||||||||||||||||||||||||||||
gliosis | an excess of astroglia (tissue consisting of large stellate (shaped like a star) neuroglial cells) in damaged areas of the central nervous system | ||||||||||||||||||||||||||||||||||
Lesion | |||||||||||||||||||||||||||||||||||
Lewy bodies | Present (cause?) | Present: Cause | whether the Lewy bodies cause or result from disease is unclear | ||||||||||||||||||||||||||||||||
neuronal loss | |||||||||||||||||||||||||||||||||||
neuropathy | Common, legs | ||||||||||||||||||||||||||||||||||
Pick bodies | Pick cells | Pick cells | abnormal neurons (Pick cells) containing inclusions (Pick bodies) | ||||||||||||||||||||||||||||||||
Tau proteins | present, but role unknown | present, role unknown | |||||||||||||||||||||||||||||||||
Tau tangles | Cause? | Often present | |||||||||||||||||||||||||||||||||
TDP-43 | present, but role unknown | ||||||||||||||||||||||||||||||||||
Functional Characteristics | |||||||||||||||||||||||||||||||||||
Abrupt onset of symptoms | Common | Common | (from: Modified Hachinski Ischemic Score) | Modified Hachinski Ischemic Score | |||||||||||||||||||||||||||||||
dystonia | acalculia | A form of aphasia characterized by the inability to perform mathematical calculations. | |||||||||||||||||||||||||||||||||
Adverse effects with antipsychotics | Common; may worsen symptoms of dementia | Common; acutely worsen extrapyramidal symptoms and may be severe or life threatening | |||||||||||||||||||||||||||||||||
Agnosia | Impairment of ability to recognize, or comprehend the meaning of, various sensory stimuli, [impaired ability to identify objects despite intact sensory function] | ||||||||||||||||||||||||||||||||||
alien hand/limb phenomenon | (difficulty controlling the movements of a limb, which seems to undertake movements on its own, sometimes combined with a feeling that the limb is not one's own) | ||||||||||||||||||||||||||||||||||
Anomia | When right side | inability to name objects | |||||||||||||||||||||||||||||||||
Anosmia | Loss of sense of smell. | ||||||||||||||||||||||||||||||||||
Aphasia | common | Impaired or absent comprehension or production of, or communication by, speech, writing, or signs [impaired ability to comprehend or use language] | |||||||||||||||||||||||||||||||||
apractic agraphia | Inability to write due to loss of knowledge about how to use a pencil or pen | ||||||||||||||||||||||||||||||||||
Apraxia | asymmetric | A disorder of voluntary movement, consisting of impairment in the performance of skilled or purposeful movements, notwithstanding the preservation of comprehension, muscular power, sensibility, and coordination in general [impaired ability to perform previously learned motor activities despite intact motor function] | |||||||||||||||||||||||||||||||||
Ataxia | balance difficulty and poor coordination | inability to coordinate muscle activity, causing jerkiness, incoordination, and inefficiency of voluntary movement | |||||||||||||||||||||||||||||||||
Atherosclerosis | Common | Common | Modified Hachinski Ischemic Score | ||||||||||||||||||||||||||||||||
Autonomic dysfunction | unexplained syncope | ||||||||||||||||||||||||||||||||||
Extrapyramidial symptoms: | Axial rigidity | occurs early | |||||||||||||||||||||||||||||||||
Behavioral disturbances | Common. wandering, agitation, yelling | late | early | Affected | Affected | cardinal manifestation | personality changes | ||||||||||||||||||||||||||||
dystonia | blepharospasm | advanced symptom | Later, possible feature | Spasmodic winking caused by the involuntary contraction of an eyelid muscle (a form of dystonia) | |||||||||||||||||||||||||||||||
Bradykinesia | Yes | Common | (63%) | A decrease in spontaneity and movement | |||||||||||||||||||||||||||||||
Cognition | Progressive decline | deficit late | deficit early | The mental activities associated with thinking, learning, and memory | |||||||||||||||||||||||||||||||
Cognitive disturbances | cardinal manifestation | ||||||||||||||||||||||||||||||||||
Consciousness | No disturbance | ||||||||||||||||||||||||||||||||||
Day-to-day fluctuation | Some | Prominent | |||||||||||||||||||||||||||||||||
Daytime drowsiness | excessive | ||||||||||||||||||||||||||||||||||
cognitive impairment | Dementia | memory loss, slowness | advanced symptom – mild-to-moderate | Later, possible feature | |||||||||||||||||||||||||||||||
Depression | Common | Common | Modified Hachinski Ischemic Score | ||||||||||||||||||||||||||||||||
dysarthria | Later (63%), monotone with slight hypophonic quality | a speech disorder caused by disturbances of muscular control because of damage to the central or peripheral nervous system. | |||||||||||||||||||||||||||||||||
dysphagia | Later | Difficulty in swallowing or inability to swallow. Also called aglutition, aphagia, odynophagia. | |||||||||||||||||||||||||||||||||
Emotional lability | Common | The property of changing readily | Modified Hachinski Ischemic Score | ||||||||||||||||||||||||||||||||
Focal neurologic deficits: | exaggeration of deep tendon reflexes | ||||||||||||||||||||||||||||||||||
Focal neurologic deficits: | extensor plantar response | ||||||||||||||||||||||||||||||||||
Focal neurologic deficits: | extrapyramidal dysfunction | Relating to or involving neural pathways situated outside or independent of the pyramidal tracts (two groups of nerve fibers arising in the brain and passing down through the spinal cord to motor cells in the anterior horns). | |||||||||||||||||||||||||||||||||
Fluctuating cognitive function | confused and unresponsive to questions (usually over a period of days) | ||||||||||||||||||||||||||||||||||
Focal neurologic signs | Common | Common | (eg, unilateral weakness, sensory loss, asymmetric reflexes, Babinski's sign) | Modified Hachinski Ischemic Score | |||||||||||||||||||||||||||||||
Focal neurologic symptoms | Common | Common | (eg, hemiparesis, homonymous hemianopia, aphasia) | Modified Hachinski Ischemic Score | |||||||||||||||||||||||||||||||
Gait | difficulty taking the first step (start hesitation) or turning. Freezing episodes | “gait apraxia” bradykinetic, broad-based, and shuffling, difficulty taking the first step (start hesitation) or turning. Freezing episodes | wider based and unstable | ||||||||||||||||||||||||||||||||
Focal neurologic deficits: | gait abnormalities | Parkinsonism, leaning to one side | |||||||||||||||||||||||||||||||||
Extrapyramidial symptoms: | gait instability | occurs early | |||||||||||||||||||||||||||||||||
Gradual decline | Gradual onset | Common | |||||||||||||||||||||||||||||||||
Grooming (hygiene) | often better-groomed and neater | ||||||||||||||||||||||||||||||||||
Hallucinations | persecutory ideation. Occur in about 20% of patients, usually when disease is moderately advanced | often benign (not threatening), when present. | common. Occur in about 80%, usually when disease is early; most commonly, visual and threatening | ||||||||||||||||||||||||||||||||
Focal neurologic deficits: | hemiplegias | Paralysis affecting only one side of the body | |||||||||||||||||||||||||||||||||
History of stroke | Common | Common | Modified Hachinski Ischemic Score | ||||||||||||||||||||||||||||||||
Hypomimia | Yes | Common | a reduced degree of facial expression | ||||||||||||||||||||||||||||||||
Hypophonia | Yes | Common | a weak voice due to incoordination of the vocal muscles | ||||||||||||||||||||||||||||||||
dystonia | impaired handwriting | Later, possible feature | Later, possible feature, tremor | ||||||||||||||||||||||||||||||||
Incontinence (fecal) | common | Later, possible feature | |||||||||||||||||||||||||||||||||
Incontinence (urinary) | Early symptom | Later, possible feature | |||||||||||||||||||||||||||||||||
dystonia | limb dystonia | Later, possible feature | |||||||||||||||||||||||||||||||||
cognitive impairment | Memory (long term) | Preserved | |||||||||||||||||||||||||||||||||
cognitive impairment | Memory (short term) | Lost early in the disease, progressive | Less affected; deficits in alertness and attention more than in memory acquisition | Preserved | common and occurs early | ||||||||||||||||||||||||||||||
Motor deficits | “Parkinsonism” early | “Parkinsonism” late | |||||||||||||||||||||||||||||||||
Myoclonus | advanced symptom | late ? | sudden, brief jerky movements | ||||||||||||||||||||||||||||||||
Nocturnal confusion | Common | Common | |||||||||||||||||||||||||||||||||
dystonia | oscillopsia | Later, possible feature | a visual sensation that stationary objects are swaying back and forth | ||||||||||||||||||||||||||||||||
Parkinsonian symptoms (Parkinsonism) | Very rare, occurring late in the disease; normal gait | Prominent, obvious early in the disease; axial rigidity and unstable gait | advanced symptom | cardinal manifestation | present | (rigidity, slow movements, postural instability) | |||||||||||||||||||||||||||||
Personality relatively preserved | Common | Common | Affected | Affected | |||||||||||||||||||||||||||||||
Progressive mental impairment | Less rapid than AD | ||||||||||||||||||||||||||||||||||
prominent neck dystonia | cardinal manifestation | [See “dystonia”] | |||||||||||||||||||||||||||||||||
Prosopagnosia | When right side | inability to recognize familiar faces | |||||||||||||||||||||||||||||||||
Focal neurologic deficits: | pseudobulbar palsy | cardinal manifestation | with pathologic laughing and crying. Appearing to be due to a bulbar (of the medulla oblongata) lesion. | ||||||||||||||||||||||||||||||||
Psychological changes | depression and anxiety, also possible: impatience, hostility, moodiness and impulsiveness | ||||||||||||||||||||||||||||||||||
REM Sleep Behavior Disorder (RBD) | Sometimes | RBD may precede the development of parkinsonism by several years | Sometimes | Sometimes | Sometimes | Sometimes | Sometimes | Sometimes | Additional degeneration of brainstem neurons is postulated to play a significant role in the control of this condition | ||||||||||||||||||||||||||
Responds to carbidopa/ levodopa | Yes | No | |||||||||||||||||||||||||||||||||
Rigidity | Common | Less common | See Parkinsonism | ||||||||||||||||||||||||||||||||
sensory loss | advanced symptom | ||||||||||||||||||||||||||||||||||
Slowing of rapid, alternating movements | Common | Less common | |||||||||||||||||||||||||||||||||
Somatic complaints | Common | (eg, arm tingling, arm clumsiness) | Modified Hachinski Ischemic Score | ||||||||||||||||||||||||||||||||
Speech | fluent but lacks meaning | ||||||||||||||||||||||||||||||||||
Stepwise deterioration | No | Common | Common | ||||||||||||||||||||||||||||||||
supranuclear ophthalmoplegia | cardinal manifestation | paralysis of the motor nerves of the eye | |||||||||||||||||||||||||||||||||
Extrapyramidial symptoms: | Symmetric deficits | no | yes | ||||||||||||||||||||||||||||||||
syncope (fainting) | unexplained episodes | ||||||||||||||||||||||||||||||||||
Transcortical dysphasia | Can repeat complex phrases but trouble with naming objects | ||||||||||||||||||||||||||||||||||
Extrapyramidial symptoms: | Tremor | occurs early | occur late | Common, early | |||||||||||||||||||||||||||||||
Tremor | Common | Less common | advanced symptom | ||||||||||||||||||||||||||||||||
urinary frequency | Early symptom | Early | |||||||||||||||||||||||||||||||||
vertical gaze palsy | most distinctive single clinical feature | ||||||||||||||||||||||||||||||||||
visual symptoms | Later (13%) | diplopia [the perception of two images of a single object], blurred vision, burning eyes, and light sensitivity | |||||||||||||||||||||||||||||||||
Visuoconstructional abilities | Deficits | affected more than other cognitive deficits | Less affected | Preserved | |||||||||||||||||||||||||||||||
Visuospatial abilities | Deficits | affected more than other cognitive deficits | Less affected | Preserved | |||||||||||||||||||||||||||||||
Weakness | |||||||||||||||||||||||||||||||||||
Focal neurologic deficits: | weakness of an extremity | ||||||||||||||||||||||||||||||||||
Word comprehension | difficulty common | progressively lost | Same as aphasia? | ||||||||||||||||||||||||||||||||
Word recall | common | ||||||||||||||||||||||||||||||||||
Focal neurologic deficits: | |||||||||||||||||||||||||||||||||||
Extrapyramidial symptoms: | Relating to or involving neural pathways situated outside or independent of the pyramidal tracts (two groups of nerve fibers arising in the brain and passing down through the spinal cord to motor cells in the anterior horns). | ||||||||||||||||||||||||||||||||||
dystonia | dyskinetic [involuntary jerky] movements due to disordered tonicity [sustained partial contraction of resting or relaxed muscles] of muscle | ||||||||||||||||||||||||||||||||||
Other/ Early Symptoms | |||||||||||||||||||||||||||||||||||
Fatigue | “vague”, usually | ||||||||||||||||||||||||||||||||||
headaches | usually | ||||||||||||||||||||||||||||||||||
Dizziness | usually | ||||||||||||||||||||||||||||||||||
Depression | usually | ||||||||||||||||||||||||||||||||||
arthralgias | usually | pain in a joint | |||||||||||||||||||||||||||||||||
personality changes | subtle, usually | ||||||||||||||||||||||||||||||||||
memory problems | usually | ||||||||||||||||||||||||||||||||||
pseudobulbar symptoms | usually | ||||||||||||||||||||||||||||||||||
unexplained imbalance or falls | cardinal manifestation (63%) | ||||||||||||||||||||||||||||||||||
Orthostatic hypotension | universal feature | Common | an excessive drop in blood pressure when the patient stands up causing light-headedness or dizziness | ||||||||||||||||||||||||||||||||
stiffness | in either the upper or lower extremities | ||||||||||||||||||||||||||||||||||
shakiness | in either the upper or lower extremities | ||||||||||||||||||||||||||||||||||
jerkiness | in either the upper or lower extremities | ||||||||||||||||||||||||||||||||||
slowness | in either the upper or lower extremities | ||||||||||||||||||||||||||||||||||
clumsiness | in either the upper or lower extremities | ||||||||||||||||||||||||||||||||||
dysphasia | less common | difficulty with speech generation | |||||||||||||||||||||||||||||||||
dysarthria | less common | difficulty with articulation | |||||||||||||||||||||||||||||||||
difficulty controlling the muscles of the face and mouth | less common | ||||||||||||||||||||||||||||||||||
walking | less common | ||||||||||||||||||||||||||||||||||
balance | less common | ||||||||||||||||||||||||||||||||||
symptom progression | usually begin on one side of the body | ||||||||||||||||||||||||||||||||||
memory | sometimes | ||||||||||||||||||||||||||||||||||
behavioral problems | sometimes | ||||||||||||||||||||||||||||||||||
Diagnostics | |||||||||||||||||||||||||||||||||||
CT (CAT) | affects the hippocampus and parietal lobes early | may [not “will”!] show bilateral multiple infarcts in the dominant hemisphere and limbic structures, multiple lacunar strokes, or periventricular white-matter lesions extending into the deep white matter | shows leukoencephalopathy in the cerebrum semiovale adjacent to the cortex, often with multiple lacunae (a small pit or hollow cavity) affecting structures deep in the gray matter | no characteristic changes | panventriculomegaly on CT or MR scan without prominent cortical atrophy | asymmetric atrophy of the fronto-parietal regions of the brain's cortex | Determine location and extent of brain atrophy. severely atrophic, sometimes paper-thin gyri in the temporal and frontal lobes. May not show the regionally prominent cortical atrophy until late | ||||||||||||||||||||||||||||
MRI | no characteristic changes | ||||||||||||||||||||||||||||||||||
PET | Positron emission tomography | ||||||||||||||||||||||||||||||||||
SPECT | single-photon emission CT | ||||||||||||||||||||||||||||||||||
Hachinski Ischemic Score | Used to differentiate between AD and VD | ||||||||||||||||||||||||||||||||||
EEG | regular, 1 per second triphasic waves | ||||||||||||||||||||||||||||||||||
Spinal Fluid | CSF drainage procedure (15-30 cc) [Some sources say 30-60?] | measure prion proteins in spinal fluid | |||||||||||||||||||||||||||||||||
Tonsil biopsy? | ??? | ||||||||||||||||||||||||||||||||||
Auditory event-related potentials | used to diff. from AD | NeuroReport 2001;12:33-37 | |||||||||||||||||||||||||||||||||
Risk Factors | |||||||||||||||||||||||||||||||||||
vascular risk factors | |||||||||||||||||||||||||||||||||||
hypertension, | |||||||||||||||||||||||||||||||||||
diabetes mellitus | |||||||||||||||||||||||||||||||||||
Average age of Onset | hyperlipidemia | after age 60 | 60 | ||||||||||||||||||||||||||||||||
Mortality / Morbidity | smoking | 6-8 years | fatal within approximately 6 years of onset, with a range of 2-17 years | ||||||||||||||||||||||||||||||||
previous strokes | |||||||||||||||||||||||||||||||||||
Other Names | VD | VD | subcortical arteriosclerotic encephalopathy | subcortical arteriosclerotic encephalopathy | DLB disease | NPH, normopressure hydrocephalus | Cortical Basal Ganglionic Degeneration, CBD, CBGD | FTD | (Multiple System Atrophy) (SDS)(MSA) | Steele-Richardson-Olszewski syndrome | Fragile X-associated tremor/ataxia syndrome (FXTAS) | Alzheimer's disease, vascular dementia, Lewy body dementia, frontal-temporal dementias, and HIV-associated dementia. Other disorders associated with dementia include Parkinson's disease, Huntington's disease, progressive supranuclear palsy, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, other prion disorders, and neurosyphilis. | |||||||||||||||||||||||
Small vessel disease | subcortical dementia | subcortical dementia | |||||||||||||||||||||||||||||||||
Related conditions | Pick's | ||||||||||||||||||||||||||||||||||
progressive supranuclear palsy | |||||||||||||||||||||||||||||||||||
Alzheimer's | |||||||||||||||||||||||||||||||||||
CNS Whipple disease | |||||||||||||||||||||||||||||||||||
Niemann-Pick type C | |||||||||||||||||||||||||||||||||||
Conditions to search: | Dentatorubropallidoluysian atrophy | frontotemporal lobar degeneration | |||||||||||||||||||||||||||||||||
Progressive pallidal atrophy | |||||||||||||||||||||||||||||||||||
CNS Whipple disease | |||||||||||||||||||||||||||||||||||
Niemann-Pick type C | |||||||||||||||||||||||||||||||||||