Flashcards in CN LANGE - Dementia & Amnestic Disorders IV Deck (89):
HIV dementia - Incidence and prevalence:
Incidence --> DOWN.
Prevalence --> UP.
HIV dementia - Risk factors:
2. Female gender.
3. Increased age.
HIV infection of the brain can produce a range of HIV-associated neurocognitive disorders:
1. Asymptomatic neurocognitive impairment (demonstrable only by cognitive testing).
2. Minor neurocognitive disorder (mild to moderate cognitive and functional impairment).
3. HIV-associated dementia (moderate to severe cognitive and functional impairment).
HIV-dementia - Pathogenesis:
Invasion of the brain by blood-borne macrophage-tropic retrovirus, which infects macrophages, microglia, and astrocytes, but NOT neurons.
HIV-dementia - The virus appears to reach the CNS ...?
Early in the course of systemic HIV-1 infection, but may remain latent and asymptomatic for years.
HIV-dementia - Can alternative produce:
Transient symptomatic HIV-1 meningitis.
HIV-dementia - Proposed mechanisms of pathogenesis:
1. Neurotoxic effects of viral proteins cytokines.
3. Other soluble factors released by inflammatory cells.
HIV-dementia - Pathology:
1. The brain in HIV-associated dementia shows perivascular infiltration by macrophages, multinucleated giant cells, and astrogliosis, which affect the basal ganglia, subcortical white matter, thalamus, and brainstem.
2. Neuronal loss may be noted in basal ganglia and frontal/temporal cortex.
3. The spinal cord may also be affected by a vacuolar myelopathy.
HIV-dementia - The onset is usually ...?
Insidious and is associated with cognitive, behavioral, and motor deficits.
HIV-dementia - Clinical findings:
1. Memory loss.
3. Difficulty with reading/writing.
4. Gait disorder.
HIV-dementia - What clinical findings have also become common with antiretroviral therapy?
1. Cortical deficits such as impaired executive function and learning.
2. Parkinsonian features.
HIV-dementia - Examination may show ...?
1. Primitive reflexes.
3. Extensor plantar responses.
4. Cerebellar ataxia.
HIV-dementia - Investigative studies - Lab test?
No definitive lab test.
HIV-dementia - CSF may show?
1. Mild to moderate elevation of protein (
HIV-dementia - MRI shows:
Cortical and subcortical atrophy with diffuse signal abnormalities in subcortical white matter and is useful for excluding other HIV-related neuropathologic processes, such as opportunistic infections.
HIV-dementia - Prognosis:
The course may be relatively static, steadily progressive, or acutely exacerbated by concurrent disease.
--> With combination antiretroviral treatment, median survival has been extended from a few months to several years.
Hematogenous spread of T.pallidum produces ...?
Signs and symptoms of 2o syphilis, including fever, skin, rash, alopecia, anogenital skin lesions, and ulceration of mucous membranes, within 1 to 6 months.
--> Neurologic symptoms are uncommon.
Earliest sign of symptomatic neurosyphilis?
Meningeal syphilis occurs ...?
2-12 months after primary infection.
Meningeal syphilis is associated with:
2. Stiff neck.
5. Cranial nerve involvement (esp. II, VII, VIII).
Meningovascular syphilis is seen ...?
4 to 7 years into the course of the disease and usually presents with transient ischemic attacks or stroke.
Late (parenchymatous) neurosyphilis produces ...?
The syndromes of general paresis and tabes dorsalis, which can occur separately OR together (taboparesis).
EITHER one can occur in combination with optic atrophy.
General paresis is a ...?
CHRONIC meningoencephalitis caused by active spirochetal infection.
General paresis - Onset is with ...?
1. Gradual memory loss or altered affect, personality, or behavior.
2. Followed by global intellectual deterioration with grandiosity, depression, psychosis, and focal weakness.
General paresis - Terminal features:
General paresis - Neurologic exam may show:
1. Tremor of the face and tongue.
2. Paucity of facial expression.
4. Pyramidal signs.
Taboparesis is the coexistence of:
Tabes dorsalis with general paresis.
Signs and symptoms of tabes dorsalis include:
1. Argyll-Robertson pupils.
2. Lancinating (stabbing) pains.
4. Posterior column sensory deficits with sensory ataxia and Romberg (+).
6. Charcot (hypertrophic) joints.
7. Genu recurvatum (hyperextended kness).
--> Optic atrophy may also be present.
Neurosyphilis - Investigative studies:
1. Treponemal serologic blood tests (fluorescent treponemal antibody absorbed [FTA-ABS]).
2. Microhemagglutination-Treponema pallidum (MHATP).
--> REACTIVE in almost all patients with neurosyphilis.
Neurosyphilis - Investigative studies - Non-treponemal blood tests (VDRL, RPR) can be ...?
NEGATIVE - Treponemal blood tests should be obtained in ALL suspected cases.
Neurosyphilis - What is needed to confirm diagnosis?
LP - Also provide a baseline CSF profile against which to gauge the efficacy of subsequent treatment.
The CSF in active neurosyphilis shows:
Lymphocytic pleocytosis + reactive non treponemal CSF serology (eg VDRL), EXCEPT in early meningeal and meningovascular syphilis + end-stage tabes dorsalis.
Neurosyphilis - Other CSF abnormalities:
1. Protein elevation.
2. Incr. gamma-globulin.
3. Presence of oligoclonal bands.
The MRI in general paresis may show ...?
Unilateral or bilateral medial temporal lobe T2 high-intensity abnormalities with or without associated atrophy.
Neurosyphilis - Treatment - Herxheimer reaction:
Transient fever and leukocytosis may occur shortly after therapy is started.
Neurosyphilis - Retreatment requirement?
Failure of the CSF to return to normal within 6 months.
Neurosyphilis - Prognosis:
General paresis may improve or stabilize after treatment, but in some cases it continues to worsen.
--> Patients with persistently reactive CSF serologic tests but no pleocytosis are unlikely to respond to penicillin therapy but are usually treated nevertheless.
PML - Results from ...?
Reactivation of JC virus infection, which is usually asymptomatic in immunosuppressed patients.
Individuals at risk for PML:
1. Lymphoma/leukemia patients.
2. HIV infection.
3. MS or Crohn disease with natalizumab or for psoriasis with efalizumab.
PML - Course:
Typically subacute and progressive, leading to death in approx. 50% of patients within 3 to 6 months, although mortality is lower in patients on natalizumab (approx. 20%).
PML associated with natalizumab or efalizumab does NOT occur until ...?
Years after treatment.
PML - Fever and systemic symptoms are ...?
PML - Symptoms/Signs:
1. Dementia and focal cortical dysfunction are prominent.
2. Signs include --> Hemiparesis, visual deficits, aphasia, dysarthria, and sensory impairment. Ataxia and headache are uncommon, and seizures do not occur.
PML - CSF is usually ...?
Normal but may show a mild incr. in:
2. WBC count.
PML - MRI/CT:
Shows multifocal white matter abnormalities.
PML - When the diagnosis is in doubt?
It can be established by brain biopsy.
PML - Treatment:
Combination antiretroviral therapy for patients with HIV and discontinuation of the drug + plasma exchange for patients receiving natalizumab.
Alcoholism as a cause of dementia:
Certain complications of alcoholism can cause dementia:
1. Hepatocerebral degeneration from alcoholic liver disease.
2. Chronic subdural hematoma from head trauma.
3. Nutritional deficiency states.
Pellagra is caused by ...?
Deficiency of niacin (nicotinic acid).
Pellagra affects the neurons of ...?
1. The cortex.
2. Basal ganglia.
5. Anterior horns of the spinal cords.
Pellagra - Systemic involvement is manifested by ...?
4. Erythematous skin lesions.
Pellagra - Neurologic involvement may produce:
3. Confusional states.
4. Pyramidal/extrapyramidal/cerebellar signs.
6. Optic neuropathy.
Pellagra - Treatment is ...?
Niacin, but the neurologic deficits may persists despite treatment.
Necrosis of the corpus callosum + subcortical white matter and occurs most often in malnourished alcoholics.
Marchiafava-Bignami syndrome - Course:
Acute, subacute, chronic.
Marchiafava-Bignami syndrome - Clinical features:
4. Gait disorder.
Marchiafava-Bignami syndrome - Diagnosis:
Can sometimes be made by MRI/CT.
Marchiafava-Bignami syndrome - Treatment:
No specific treatment is availables - But cessation of drinking and improvement of nutrition are advised.
Marchiafava-Bignami syndrome - Prognosis:
1. Patients may die.
2. Survive with dementia.
Alcoholic dementia is caused by ...?
Direct toxic effects of ethanol on the brain has been proposed to occur, but no distinctive abnormalities have been identified in the brains of demented alcoholics.
Dementia in alcoholics is more likely the result ...?
Of one of the metabolic and traumatic disorders mentioned previously, OR misdiagnosis of Korsakoff syndrome.
Hypothyroidism - Dementia:
Hypothyroidism can also produce a REVERSIBLE dementia OR chronic organic psychosis.
The dementia in hypothyroidism is ...?
Global disorder characterized by:
1. Mental slowness.
2. Memory loss.
--> Focal cortical deficits do not occur.
Dementia in hypothyroidism - Psychiatric symptoms:
3. Visual and auditory hallucinations.
5. Suicidal behavior.
Patients with myxedema - Examination may show:
3. Cerebellar ataxia.
--> MOST suggestive finding is delayed relaxation of the tendon reflexes.
B12 def. - Dementia may occur with or without ...?
Hematologic and other neurologic manifestations (although diminished vibratory sensation and proprioception in the lower extremities are common.
B12 def. - The dementia consists ...?
1. Global cognitive dysfunction with mental slowness, impaired concentration, memory disturbance.
2. Aphasia and other focal cortical disorders do NOT occur.
B12 def. - Psychiatric symptoms:
3. Paranoid psychosis with visual and auditory hallucinations.
Dialysis dementia - Clinical features:
--> Initially intermittent, but later become permanent, and dementia supervenes.
Dialysis dementia - EEG:
Shows paroxysmal high-voltage slowing with intermixed spikes and slow waves, which can be reversed by diazepam.
What has decreased the incidence of dialysis dementia?
Removing trace metals from the diasylate.
Cognitive dysfunction and associated subcortical white matter lesions on MRI may also be associated with ...?
Recurrent episodes of cerebral hypoperfusion during hemodialysis.
Non-Wilsonian hepatocerebral degeneration:
Uncommon complication of chronic hepatic cirrhosis with spontaneous or surgical portosystemic shunting.
Non-Wilsonian Hepatocerebral degeneration - Symptoms may be related to failure of the liver to ...?
--> Neurologic symptoms may precede hepatic symptoms in approx. 1/6 of patients.
Non-Wilsonian hepatocerebral degeneration - Clinical findings - The neurologic syndrome ...?
Is fluctuating but progressive over 1 to 9 years and may be punctuated by episodes of acute hepatic encephalopathy.
Non-Wilsonian hepatocerebral degeneration - Clinical findings - Common features:
3. Cerebellar/extrapyramidal/pyramidal signs.
Non-Wilsonian hepatocerebral degeneration - Clinical findings - Dementia is marked by ...?
1. Mental slowness.
3. Impaired attention.
5. Memory disturbance.
Non-Wilsonian hepatocerebral degeneration - Clinical findings - Cerebellar signs:
1. Gait and limb ataxia.
3. Nystagmus is rare.
Non-Wilsonian hepatocerebral degeneration - Clinical findings - Extrapyramidal involvement may produce:
2. Resting tremor.
Non-Wilsonian hepatocerebral degeneration - Clinical findings - Common signs:
4. Extensor plantar responses.
--> Paraparesis is rare.
Non-Wilsonian hepatocerebral degeneration - Lab studies:
1. Abnormal LFTs.
2. Elevated blood ammonia.
--> The degree of abnormality bears NO DIRECT relationship to the severity of neurologic symptoms.
Non-Wilsonian hepatocerebral degeneration - MRI:
May show lesions in the basal ganglia and subcortical white matter.
Non-Wilsonian hepatocerebral degeneration - Lab studies - CSF:
Normal, except for increased glutamine and occasional mild elevation of protein.
Non-Wilsonian hepatocerebral degeneration - DDx:
1. Wilson disease --> Earlier onset, K-F rings, abnormal copper metabolism.
2. Alcoholic cerebellar degeneration primarily affects gait and is NOT accompanied by extrapyramidal or pyramidal signs.
Non-Wilsonian hepatocerebral degeneration - Treatment:
1. Low-protein diet.
4. Liver transplantation.
5. Portosystemic shunting.
6. Improvement after levodopa or bromocriptine therapy has been described!
Non-Wilsonian hepatocerebral degeneration - Death usually results from ...?
Progressive liver failure or variceal bleeding.
Pseudodementia - Which condition is most commonly mistaken for dementia?
Both dementia and depression can be characterized by ...?
1. Mental slowness.
6. Difficulty with memory and concentration.
7. Changes in behavior and personality.