Behavior potpourri

Question 1

transcortical motor aphasia

Answer 1

A transcortical motor aphasia is a nonfluent aphasia with preserved naming and repetition, differentiating it from a Broca aphasia, which has impairment of spontaneous speech, naming, and repetition. The most common neurologic symptom is a right hemiparesis that predominately affects the leg. The lesion producing a transcortical motor aphasia lies in the left frontal lobe and is usually caused by a stroke of the anterior cerebral artery. Sensory loss is usually absent or restricted to the right leg and visual field deficits are usually absent.

Question 2

treatments of Tourette’s disorder

Answer 2

Pimozide and haloperidol are approved and the most effective treatments of Tourette’s disorder.

Question 3

symptoms of herpes encephalitis

Answer 3

Symptoms of herpes simplex encephalitis include anosmia, olfactory and gustatory hallucinations, and personality changes that can involve bizarre and psychotic behaviors.

Question 4

symptoms of rabies encephalitis

Answer 4

Symptoms of rabies encephalitis include agitation, restlessness and hydrophobia.

Question 5

symptoms of neurosyphilis

Answer 5

Symptoms of neurosyphilis personality change, poor judgement, delusions of grandeur, poor self-care.

Question 6

symptoms of crypto meningitis

Answer 6

Symptoms of cryptococcal meningitis include headache, memory impairment and confusion.

Question 7

symptoms of lyme disease

Answer 7

Symptoms of Lyme disease include irritability, depression, poor concentration and memory impairment.

Question 8

biomarkers for AD

Answer 8

The Alzheimer disease neuroimaging initiative (ADNI) was established to look for biomarkers to assist in the diagnosis and treatment of Alzheimer disease (AD) and mild cognitive impairment (MCI). Numerous studies have evaluated the cerebrospinal fluid profiles of normal controls, patients with MCI who progressed to dementia, stable MCI, and Alzheimer disease. The presence of increased phospho-tau levels, in conjunction with decreased AB42 levels, is most closely associated with conversion from MCI to AD. AB42 levels are also most sensitive to functional decline in patients with AD.

Question 9

stroop test

Answer 9

In the Stroop test, names of colors are spelled in different color ink and the patient is asked to name the ink color although the actual word spelled may be different than the ink color. For example, the word RED is spelled in blue ink and the patient needs to be able to suppress the actual printed word they see and say “blue.”

Question 10

kluver-bucy syndrome

Answer 10

This syndrome is known as Klüver-Bucy syndrome and occurs as a result of bilateral temporal lesions involving amygdalae. Hypermetamorphosis, a common feature, involves excessive fascination with objects in the patient’s environment and the urge to touch them all.

Question 11

balint syndrome

Answer 11

Balint’s syndrome is composed of the triad of optic ataxia (the inability to reach for an object under visual guidance), simultanagnosia (the inability to view multiple objects at once), and ocular apraxia (the inability to voluntarily direct gaze to a specific target). It usually results from bilateral damage to the parietal-occipital region secondary to separate infarctions. However, cases have been reported following head trauma as well as neoplastic disease.

Question 12

Structural or functional lesions in the _______ are more often associated with depression than other areas.

Answer 12

left anterior frontal lobe

Question 13

PET using _______ is currently the most specific in vivo reflection of amyloid-beta protein load.

Answer 13

Pittsburgh compound-B

Question 14

TDP-43

Answer 14

Transactivating responsive sequence DNA-binding protein (TDP-43) is a protein encoded by the TARDBP gene on chromosome 1. Numerous studies have linked this protein to the syndromes of amyotrophic lateral sclerosis, frontotemporal dementia with motor neuron disease and frontotemporal dementia with ubiquitin inclusions.

Question 15

mutation corresponding to FTD

Answer 15

Mutations in the progranulin gene can cause frontotemporal dementia

Question 16

Parkinson’s disease and dementia with Lewy bodies are associated with abnormal ___

Answer 16

synuclein

Question 17

Huntington mutation

Answer 17

Abnormality of the huntington gene on chromosome 4p16.3 results in Huntington disease.
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Question 18

The impairment in motor programming with insufficient rhythm tapping, set shifting, and go/no-go tasks is typically seen in injuries of the _____.

Answer 18

dorsolateral frontal regions

Question 19

aphemia

Answer 19

Aphemia, also known as apraxia of speech, is characterized by occasional mutism, diminished fluency, and deficits in articulation. Despite appearing to have nonfluent aphasia, patients with aphemia have retained the ability to write.

Question 20

Hashimoto encephalitis

Answer 20

In this condition, TSH and T3/T4 levels are frequently normal; however, anti-microsomal antibodies are typically elevated. This condition should be in the differential of any patient with a rapidly progressive dementia, especially in the presence of seizures. Hashimoto encephalitis responds well to intravenous corticosteroid treatment and plasmapheresis.

Question 21

alexia without agraphia

Answer 21

If the splenium of the corpus callosum was damaged, it can cause a pure alexia without agraphia due to the right occipital lobe’s inability to send visual information to the language centers of the brain on the left. This patient is able to read and write.

Question 22

Simultagnosia

Answer 22

Simultagnosia, or the inability to perceive more than one item or feature at a time, is usually caused by bilateral lesions to the occipitoparietal lobes. When combined with optic apraxia and ocular ataxia, it is more commonly known as Balint syndrome.

Question 23

Treatment of parkinson’s with hallucinations or delusions

Answer 23

Quetiapine is an effective antipsychotic with very little dopaminergic blocking activity; hence, it is useful in Parkinson disease patients with hallucinations or delusions. It acts predominantly on serotonin receptors. Risperidone, perphenazine, and haloperidol all have more dopamine blocking properties than quetiapine. Chlorpromazine carries a risk for orthostatic hypotension and potential for falls. Olanzapine has more extrapyramidal and anticholinergic properties than quetiapine.

Question 24

dementia drugs

Answer 24

• Rivastigmine is FDA approved to treat mild to moderate Parkinson and Alzheimer disease.
• Donepezil is FDA approved for treatment of mild to severe Alzheimer disease.
• Galantamine is FDA approved for mild to moderate Alzheimer disease.

Question 25

conduction aphasia

Answer 25

Typical features of conduction aphasia include frequent paraphasic errors, impaired repetition, spontaneous verbal output but fair to good comprehension and only mild impairment in fluency.

Question 26

serotonin syndrome

Answer 26

The serotonin syndrome results from concomitant administration of medications that enhance serotonin transmission via decreased breakdown, or increased production. Medication combinations to use cautiously include monoamine oxidase inhibitor agents with selective serotonin reuptake inhibitors, tricyclic antidepressants, or dextromethorphan. The serotonin syndrome can be differentiated from neuroleptic malignant syndrome by the presence of shivering and myoclonus in the former.

Question 27

neuro-sarcoid

Answer 27

Neurologic involvement can occur in 5% to 10% of patients with sarcoidosis. Manifestations can include motor and sensory deficits, progressive visual loss, and cranial nerve palsies. Cognitive impairments can range from mild memory loss to progressive dementia. Sarcoidosis appears to have a predilection for the hypothalamus and basal forebrain resulting in hypopituitarism, hyperphagia, and hypersomnolence.

Question 28

dementia with lewy bodies

Answer 28

The dementia with Lewy bodies (DLB) consortium has revised criteria for the clinical and pathologic diagnosis of DLB, incorporating new information about the core clinical features and suggesting improved methods to assess them. REM sleep behavior disorder, severe neuroleptic sensitivity, and reduced striatal dopamine transporter activity on functional neuroimaging are given greater diagnostic weighting as features suggestive of a DLB diagnosis. When any of these features are present with one of the primary findings of visual hallucinations, parkinsonism, or fluctuating attention, then the diagnosis of probable DLB is supported.

Question 29

Alien Limb Syndrome

Answer 29

seen in corticobasal degeneration (CBD), referable to the parietal lobe

Question 30

Pathology of AD

Answer 30

CSF level of beta-amyloid (1-42) is decreased due to what is thought to be a reduction of clearance of beta-amyloid from the brain to the CSF/blood as well as aggregation and plaque deposition in the brain, further reducing the amount found in the CSF. intraneuronal inclusions of the microtubule-associated protein tau. Tau is markedly hyperphosphorylated in Alzheimer’s dementia and the level in the CSF is also very high and can act to differentiate Alzheimer’s dementia from other forms of dementias.

Question 31

Logopenia

Answer 31

Logopenia is considered when there is low word production with intact content.

Question 32

falls in patient’s with neurodegenerativer parkinsonisms

Answer 32

While many patients with parkinsonism can fall, patients with progressive supranuclear palsy (PSP) have truncal/axial rigidity and tend to fall backwards as opposed to the appendicular rigidity seen in the other neurodegenerative disorders presented here. Spilling food is a common presentation of PSP due to an inability to look down while eating. PSP and corticobasal degeneration are both associated with frontotemporal dementia and obsessive compulsive behaviors, but corticobasal degeneration has appendicular rigidity with forward and side falls. Falls in multiple system atrophy may be due to dysautonomia, but would not explain the other clinical symptoms.

Question 33

west nile encephalitis

Answer 33

Encephalitis caused by the West Nile virus (WNV) generally happens in older patients and presents with mental status changes and prominent movement disorders, secondary to a predilection for the basal ganglia and thalamus. WNV can also cause a flaccid paralytic syndrome, similar to the polio virus.

Question 34

Williams syndrome

Answer 34

Williams syndrome is a neurodevelopmental disorder caused by deletions on chromosome 7. Patients are generally very friendly with excellent social and language skills. Marked impairment is noted on tests of visual processing, affecting both the dorsal and ventral streams. Nonneurologic symptoms include cardiovascular abnormalities, hyperacusis, endocrine disorders, and hypercalcemia.

Question 35

landau-kleffner syndrome

Answer 35

Landau-Kleffner syndrome (acquired epileptic aphasia) should be differentiated from cases of autism with minimal language regression, especially when associated with isolated EEG abnormalities. Acquired epileptic aphasia typically develops in healthy children, who acutely or progressively lose receptive and expressive language ability, coincident with the appearance of paroxysmal EEG changes, usually in sleep. Rasmussen encephalitis has epilepsia partialis continua.

Question 36

cholinesterase inhibitors side effects

Answer 36

Typical cholinesterase inhibitor (donepezil, rivastigmine, and galantamine) side effects include nausea, vomiting, and diarrhea. Even after prolonged treatment, patients can later develop GI side effects leading to poor PO intake, weight loss, and a failure to thrive phenotype. Cholinesterase inhibitors are basal forebrain activators which can lead to vivid nightmares. These medication are also nodal blockers and can lead to bradycardia and syncopal events over time. Finally, donepezil can worsen depression symptoms: asthenia, fatigue, and somnolence.

Question 37

FTD with motor neuron disease

Answer 37

this man best fits the diagnosis of frontotemporal dementia (FTD) with motor neuron disease. This condition is characterized by personality changes prior to memory disturbances typically before age 65 and development of upper and lower motor neuron deficits (Amyotropic Lateral Sclerosis). An abnormal expansion of a hexanucleotide repeat in the gene C9orf72 (Chromosome 9 open reading frame 72) is the most common genetic cause of both FTD and ALS resulting in TDP-43 protein inclusions.

Question 38

Genetics and Dementia

Answer 38

Mutation in the alpha-synuclein gene is seen in families with Parkinson’s disease. Notch 3 gene mutations are the cause of CADASIL. MAPT (tau) gene mutations lead to certain forms of familial FTD but only rarely in those who also have ALS. Presenilin 1 gene mutations typically cause AD.