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Question 1

What are the links in the Circuit of Papez

Answer 1

The circuit of Papez is: entorhinal cortex → hippocampus → fornix → mammillary bodies → anterior nucleus of thalamus → cingulate gyrus → entorhinal cortex → hippocampus.

Question 2

What are features of an orbitofrontal lesion?

Answer 2

Symptoms of orbitofrontal lesions may include traits of obsessive compulsive disorder (OCD), disinhibition, hypersexuality, anxiety, depression, impulsiveness, and antisocial behavior.

Question 3

Familial FTD - genetic link?

Answer 3

In familial frontotemporal dementia (FTD), the most common linkage is to chromosome 17q21,

Question 4

Syndrome of bilateral ACA infarcts?

Answer 4

bilateral anterior cerebral artery (ACA) infarcts. The key is identifying symptoms of frontal lobe dysfunction (such as those listed in the question) and leg weakness

Question 5

A lesion of the dorsomedial nucleus would cause what symptoms?

Answer 5

The dorsomedial nucleus is in the thalamus and has projections to dorsolateral prefrontal, orbitofrontal, anterior cingulate gyrus, and temporal lobe/amygdala. Dysfunction of this nucleus can result in abulia, anterograde amnesia, social disinhibition, and motivation loss

Question 6

Huntington’s disease is associated with mutation on which chromosome?

Answer 6

Huntington’s disease is an autosomal dominant trinucleotide repeat disorder resulting from expansion of CAG repeats on chromosome 4p in a region that codes for the Huntington’s protein. The disease is associated with choreoathetosis and dementia.

Question 7

What is Kluver Bucy syndrome?

Answer 7

Kluver–Bucy syndrome is caused by lesions to bilateral anterior temporal lobes/amygdala and is characterized by hyperorality (tendency to explore objects with mouth), hypermetamorphosis (preoccupied with minute environmental stimuli), blunted emotional affect, hypersexuality, and visual agnosia. It has been associated with Pick’s disease

Question 8

Compare the trigeminal autonomic cephalgias: duration of attacts, associated features, recommended abortive medications, recommended preventive medications?

Answer 8

SUNCT: 1 - 240 second attacks. SUNA without conjunctival injection and or tearing - stabbing headache quality, opthalmic distribution o fpain. Abortive medications often unnecessary. Lamotrigine for preventative. Paroxysmal hemicrania: 2-30 minutes: high daily attack frequency (>5 per day) absence of agitiation. Abortive medications often unnecessary as good response to indomethacin (use for prevention) Cluster headache: 15-180 minutes: more prevalent in males: circadian or circannual recurrence, significant associated agitation, triggered by alcohol. Can try subcutaneous sumitriptan, intramuscular DHE, high flow O2 to abort To prevent- verapamil, lithium, topiramate Hemicrania continua - 30 mins to 3 days: baseline milder headache for > 3 months attacks with automonic features. Often unnecessary given good response to indomethacin to use abortive but use indomethacin at high doses for preventative treatment.

Question 9

Genetic mutatons and location on chromosomes for AD, FTD,

Answer 9

AD: preselin 1 (ch14) and preselin 2 (ch1), APP (ch 21) FTD: GRN Ch 17, MART CH 17, C9Orf72 (ch 9)

Question 10

Frontal Lobe Signs

Answer 10

• Utilization behaviour: uses something in the environment automatically
• Failure of response inhibition: hallmark of orbital frontal dysfunction

Question 11

Manifestations of Tropheryma whippelii

Answer 11

• Whipple disease.
• Patients typically present with gastrointestinal complaints including diarrhea, malabsorption and weight loss.
• Other symptoms may include lymphadenopathy, hyperpigmented skin, movement disorders, oculomasticatory myodysrhythmia and dementia.

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Question 12

CJD

Answer 12

• Sporadic CJD
• 85-90% of cases
• 6 clinical phenotypes:
• Familial CJD disease is seen in 10-15% CJD of cases. Clusters of CJD cases are usually familial.
• Transmitted (iatrogenic) CJD can occur via the transfer of human tissues, CSF, or blood contaminated with prion proteins (e.g. corneal transplant, neurosurgical equipment without sufficient sterilization, administration of contaminated growth hormone, or blood). These cases are very rare.
• Variant CJD (vCJD) is also a rare form caused by dietary consumption of beef that contains brain or spinal cord tissue infected with the prion responsible for bovine spongiform encephalopathy (BSE).
• Trauma can lead to Chronic Traumatic Encephalopathy (CTE), a progressive neurodegenerative condition that has a very slow progression over years.

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Question 13

CJD IMAGING

Answer 13

• MRI has been shown to have higher specificity and sensitivity than EEG and CSF testing for 14-3-3 protein.
• MRI: DWI/ADC showing hyperintensity in the cortical gyri, caudate, and thalamus.

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Question 14

vCJD

Answer 14

• Young (av 28). Course of illness 1 year
• Anxiety, depression withdrawal sx, Persistent pain and abnormal sensation in the face and limbs, Over several months then develop myoclonus, gait disturbance, dementia
• Prion proteins cause spongiform encephalopathy characterized by rapidly progressive dementia with myoclonus and seizures.
• WHAT DOES THE MRI SHOW? WHAT IS THE HISTOPATH?
• MRI – pulvinar
• Neuropathologically the most conspicuous pattern is the formation of multiple amyloid plaques surrounded by a rim of spongiform change (florid plaque) which ranges from size of 5-50mm and has a hyaline eosinophilic core surrounded by a paler halo is seen on haematoxylin and eosin staining, Similar smaller plaques are seen in clusters in perivascular and pericellular arrangements. Florid plaques are characteristic but not specific for vCJD

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Question 15

WHAT IS THIS - Pulvinar sign: T2 hyperintensities in the thalamus, basal ganglia and cortex, and path – florid plaque

Answer 15

VARIANT CJD

Question 16

Catatonia

Answer 16

• Immobility and rigid motor tone and “waxy flexibility” (when you change a limb position patient retains that position for up to days)
• Commonly associated conditions: schizophrenia, mood disorders, general medical conditions
• There are more than 40 diagnostic criteria for catatonia but can be confident with 2 of 1. motoric immobility (either waxy flexibility or stupor) 2. excessive purposless motor activity, 3. extreme negativism or mutism 4) peculiar movements including posturing, mannerisms or grimmacing, 5) echolalia or echopraxia
• Think: immobility, stupor, posturing, staring, rigidity, grimacing, withdrawal, mute, echopraxia, echolalia, sterortypies and mannerisms
• WHAT IS THE MOST COMMONLY ASSOCIATED DISORDER
• Bipolar I (mood disorders (25-50%), schizophrenia (10-15%)
• Treatment: sedative anticonvulsants (barbiturates and benzos) or ECT

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Question 17

MCI

• Essentially - its greater memory impairment than expected for age but not enough to affect function
• What % progress to Dementia?
• What can be effective in preventing progression to dementia?
• What is predictive on CSF

Answer 17

• Donepezil can slow the progression of MCI into Alzheimers in the first 12 months of treatment
• Rate of conversion of MCI to Alzheimer’s per year is 16% per year
• Cerebrospinal fluid (CSF) raised tau and lowered beta amyloid 1-42 Tau - levels released from dying neurons, beta-amyloid 1-42 are decreased secondary to the accumulation of amyloid in the neuritic plaques - This pattern has been shown to have sensitivity and specificity of 85% in predicting conversion from mild cognitive impairment (MCI) to AD.

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Question 18

Order of investigation for dementia

Answer 18

• 1. brief cognitive assessment
• 2. collateral
• 3. bloods and screening CT/MRI

Question 19

Bloods – impaired cognition

Answer 19

• B12 deficiency: likely to have decreased haemaglobin, elevated MCV, combined system degeneration (posterior columns and lateral corticospinal tract signs)
• Wilson’s disease: LFT derrangements, movement disorder
• Hypothyroidism: depressed relaxation phase of reflexes,
• Niacin deficiency, (think if taking isoniazid) dementia, dermatitis, and diarrhea.
• Vitamin E deficiency typically presents with cognitive dysfunction, ataxia, and neuropathy.

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Question 20

Diagnosing AD

Answer 20

• NINCDS-ADRDA : onset 40-90 progressive cognitive loss with deficits in 2 + domains
• Probable- established by clinical examination and confirmed by neuropsychologicaltests
• Possible: having dementia syndrome in absence of other psyvh illness, but in presence of a second systemic or brain disorder not thought to be the cause of your problems
• Definite: clinical criteria for probable plus histopathologic evidence (autopsy or biopsy)

Question 21

MRIs and PETs - FINDINGS IN AD, LBD, FTD

Answer 21

• AD: hypometabolism in bilateral parietal and precuneus.
• LBD: Bilateral parietal-occipital hypometabolism
• FTD: bilateral frontotemporal

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Question 22

Cholinesterase Inhibitors - SE, MOA

Answer 22

• SE: bradycardia – heart problems relative CI
• Other CI – gastric ulcer bleeds, seizures, bronchial muscle spasm secondary to asthma or COPD
• Acetylcholinesterase inhibitors can reduce neuropsychiatric symptoms (including psychosis) in patients with Alzheimer’s disease, Parkinson’s disease dementia and diffuse Lewy body disease.
• LBD: most evidence for Rivastigmine

Question 23

Test Types

Answer 23

Question 24

TEST TYPES

Answer 24

Question 25

Testing What is digit spam testing and what is delayed recall testing

Answer 25

* Digit span, which involves attentional processes, immediate recall (active working memory) and ability to sequence bits of information, may be reduced following lesions of dorsolateral frontal cortex. * Memory (delayed recall) Lesions of the fornix, hippocampus, mammillary bodies and medial dorsal nucleus of the thalamus

Question 26

DMS classifications

Answer 26

* DSM-IV Axial Categories * n Axis I: psychiatric ‘state’ conditions * n Axis II: personality disorders and mental * retardation – ‘trait’ conditions * n Axis III: all medical/neurologic conditions * such as HTN, DM, epilepsy * n Axis IV: psychosocial stressors – divorce, * domestic violence, unemployment * n Axis V: global assessment of function * (GAF) score: 1-100 •

Question 27

Operant Conditioning Definitions

Answer 27

* Operant Conditioning Definitions * Reinforcement: a consequence that causes a behavior to increase * Punishment: a consequence that causes a behavior to decrease * Negative: subtraction of a stimulus * Positive: addition of a stimulus * Extinction: no change in consequence causes a decrease in behavior •

Question 28

What is the Clinical Dementia Rating Scale – At what rating is driving contraindicated

Answer 28

* CDR numeric scal to quantify severity of dementia * Cognitive and functional testing in 6 areas: memory, orientation, judgement & problem solving, community affairs, homes and hobbies, personal care. * 0: no dementai * 0.5 very mild dementia * 1: mild dementia * 2: moderate * 3: severe * DRIVING: 1 or greater have substantially increased accident rate and thus should not be able to drive. 0.5-evaluation should be considered and readressed every 6 months

Question 29

Which is the best scale for AIDS dementia testing?

Answer 29

* Wisconsin card sorting test * Compared to Neurlogically intact AIDS subjects, patients with mild AIDS dementia complex are markedly worse in executive function, memory, and complex attention, but not in orientation, language, or visuospatial construction.

Question 30

HIV encephalopathy: what to know

Answer 30

* HIV encephalopathy (or AIDS dementia complex) is a subacute inflammatory infiltration fo the brain caused by direct spread of HIV to the CNS) * Cognitive impairment, psychomotor delay, incontinence, impaired motor skills, confusion * CSF and MRI normal

Question 31

Apraxia: corpus callosum stroke – where is the lesion?

Answer 31

* Apraxia can occur with lesions of the corpus callosum * Corpus callosum contains crossing fibers from dominant hemisphere to premotor cortex * Hand apraxia with corpus callosum stroke represents a classid disconnection syndrome * Patients are typically apractic only in their non dominant hand

Question 32

Ring enhancing lesion with oedema in 50, non – immunosuppressed – what is most likely?

Answer 32

* Glioblastoma multiforme * Symptoms of less than 3 months duration * DDx metastatis and abscess

Question 33

What is the most common psychiatric disorder in patients with Epilepsy?

Answer 33

* Depression * The relationship between epilepsy and depression is thought to be bi-directional * Over half of epileptics have one or more episodes of significant depression during the course of the disorder and the suicide rate is considerably greater than in the general population. Slightly over 20% have had active current or past year depression. Episodic dyscontrol is not a psychiatric disorder. * Forced normalization refers to a psychosis occurring after achievement of good clinical seizure control, or resolution of interictal epileptiform discharges. • •

Question 34

Progressive Aphasias

Answer 34

* Logopenic progressive aphasia (LPA) - Hypometabolism is seen in the left temporal/parietal regions * semantic dementia (FTD) - Bilateral anterior temporal hypometabolism, have increased difficulty understanding nouns and recognising objects, can develop visual agnosia and prosopagnosia too. Patients with semantic dementia lose conceptual knowledge of memories and words. They tend to have frequent pauses in their speech and can present with prosopagnosia. * progressive anarthria, * progressive nonfluent aphasia - left frontal opercular region

Question 35

Speech: impaired comprehension, with intact repetition,

Answer 35

* Transcortical sensory aphasia * Lesion surrounding wernicke’s area * Watershed infarct between MCA and PCA (posterior parieto-occipital area) * Doesn’t involve the deeper arcuate fasciculus * Naming difficulties

Question 36

Non fluent, with repetition intact

Answer 36

* Transcortical Motor aphasia * ACA-MCA watershed infarct, * Doesn’t involve the deeper arcuate fasciculus * Naming difficulties * Transcortical motor aphasias usually involve an infarct of the supplementary motor area and adjacent cingulate gyrus in the distribution of the anterior cerebral artery. •

Question 37

Conduction Aphasia

Answer 37

* A pure lesion of the arcuate fasciculus * Can speak fluently * Comprehension is intact * Unable to repeat even simple phrases * Reading and writing preserved * Naming difficulties

Question 38

Broca’s

Answer 38

* Non fluent (motor) aphasia * Difficulty with word output * Comprehension is intact * Repetition can be impaired * Posterior lateral frontal lobe, close to the homunculus for face and arm * Naming impaired * Agrammatism (inability to speak in a grammatically correct fashion) with simplified formation of sentences

Question 39

Anomic Aphasia

Answer 39

* Will have impaired naming * Repetition will be intact

Question 40

Wernicke’s

Answer 40

* Fluent (or sensory) aphasia * Comprehension difficulty * Spontaneous speech demonstrates word substitutions and paraphasia * Fluidity of speech Is preserved * Repetition can be impaired * Naming impaired * Neologisms are made up words seen often in Wernicke's aphasia.

Question 41

Aphemia

Answer 41

* Pure word mutism * Dominant frontal lobe lesion * Complete inability to speak but preserved comprehension and ability to read and write * May be associated R face and arm weakness

Question 42

Amusia

Answer 42

•Inability to appreciate music and is caused by a lesion in th enon-dominant hemisphere

Question 43

Aprosodia

Answer 43

* Lack of musicality of speech, unable to produce melodic inflection in speech, loss of nonverbal affective expression * Lesion in the non dominant hemisphere – posterior inferior frontal lobe right * Anologous to brocas area on the dominant side

Question 44

Pure word deafness

Answer 44

* inability to understand spoken language * hearing isintact. * spontaneous speaking, reading, and writing are preserved. * This is most often seen in patients who have lesions of the superior temporal gyrus bilaterall • •

Question 45

Which type of dementia is associated with the development of new muscial and artistic abilities?

Answer 45

* Frontotemporal dementia * Apathy, disinhibition, planning and organizing difficulties

Question 46

Pick’s Disease

Answer 46

* Pick’s disease is one of the FTDs * Presents with behavioural changes and executive function changes * Imaging: frontal and temporal lobe atrophy

Question 47

What are the pathogenic features of the dementias?

Answer 47

Question 48

Pathology findings AD

Answer 48

* Neuritic plaques * Neurofibrillary tangles * Amyloid angiopahty * Granulovacuolar degeneration consists in small intraneuronal cytoplasmic vacuoles, each of which contains an argyrophilic granule

Question 49

AD Genetics Chromosome’s

Answer 49

* Familial forms of AD accound for \< 7% of all cases of AD * The most common genetic mutation associated with familial AD is presenilin 1 mutation * Mutations of Presenilin 1 (on chromosome 14) and Presenilin 2 (on chromosome 1) are linked to Familial AD * Down syndrome (trysomy 21) is associated with early AD * Amyloid Precursor Protein gene – thought to be pathogenic is located on chromosome 21. * Susceptibility gene for general AD * Not sufficient nor necessary to cause the disease * APOE gene is the most common susceptibility gene linked to Alzheimer disease (AD). * ]APOE 4 allele will increase the risk of developing the disease between 5- and 15- fold; * (Presenilin and APP mutations will independently cause disease and therefore are not considered susceptibility genes).

Question 50

Anosagnosia

Answer 50

•Anosagnosia is the real or feigned ignorance of the presence of disease, especially of paralysis.

Question 51

ANTON Syndrome

Answer 51

* Denial of blindness despite objective evidence of visual loss * Typically associated with posterior cerebral artery territory infarction producing cortical blindness plus memory impairment

Question 52

Associative visual agnosia

Answer 52

•the syndrome is seen in left occipitotemporal junction lesions. The majority of patients will also have a dense right homonymous hemianopia. •

Question 53

Personality Disorders:

Answer 53

* Cluster A: odd and eccentric: Schizoid, Schizotypal and Paranoid * Cluster B: dramatic: borderline, histrionic, antisocial, narcissistic * Cluster C: fearful/anxious: avoidant, dependent, obsessive-compulsive

Question 54

Topographagnosia: what is it, where is the lesion?

Answer 54

* Topographagnosia is the inability to find one’s way around, read maps, draw plans and perform similar functions * Right (non dominant) parietal lobe

Question 55

Key features for Autism / Asperger’s

Answer 55

* Severe and sustained impairment in social interaction * Restricted development * Repetitive patterns of behaviour, interest, activities, causing clinically signigicant impairment in social, occupational or other areas of functioning * There are NO other clinically significant delays in language, no other delays in cognitive development, or environmental curiosity * Testing: Routine genetic testing is recommended for all children with autism spectrum disorder. Brain imaging, EEG and CSF analysis are not routinely indicated unless there is a clear history of regression, or there are focal deficits or possible seizure like episodes. •

Question 56

Asperger’s vs Childhood disintergrative disorder

Answer 56

* Childhood disintegrative disorder (CDD) is characterized by late onset (\>2 years of age) of developmental delays in language, social function, and motor skills. * Asperger syndrome differs from childhood disintegrative disorder by its relative preservation of linguistic and cognitive development. Both show no delay in acquiring initial language skills but after age 2, those with CDD regress in their language and communications skills. Both of these developmental disorders display impairment in social functioning skills and show repetitive behavior and restricted interests. •

Question 57

Non dominant parietal

Answer 57

* agraphesthesia * astereognosis (left) * decreased 2-point discrimination (left).

Question 58

Prosopagnosia

Answer 58

* Inability to recognise faces * Fusiform gyrus – also known as the occipitotemporal gyrus

Question 59

Faking It

Answer 59

* Factitious disorder:intentionally act as physically or mentally ill without obvious benefits – often have borderline personality disorder * Malingering: intentionally acts ill with obvious secondary benefits from the sick role * Ganser syndrome: factitious disorder or a dissociative disorder, characterized by pseudo-stupidity- the syndrome of approximate answers, Patient gives wrong or silly answers but approximate enough to the question to indicate they understood. * Conversion – physical symptoms produced by unconscious psychological issues * Munchausen syndrome by proxy: parent presents with a complaint with the child * Amnestic: difficulty remembering new things * Somatiform: individuals with have recurrent and multiple somatic complaints not due to any physical disorder

Question 60

Reduplicative paramnesia

Answer 60

* The delusional belief that the place or location has been duplicated, existing in two or more sites simultaneously. * Most commonly associated with acquired brain injury * Particularly right parietal and bi frontal

Question 61

Landau Klefner Syndrome: think language regression and seizures

Answer 61

* Acquired Epileptic Aphasia * Usually develops in healthy children who acutely or progressively lose receptive and expressive language ability and have concomitant new onset paroxysmal EEG changes * Clinical seizure prevalence 70-85% * Aphasia usually appears by 4-7 * Receptive language primarily affected

Question 62

Schizophrenia: MRI findings?

Answer 62

•Studies have showed schizophrenia is associated with bilateral ventriculomegaly and medial temoral area decreased brain volume.

Question 63

Management of ADHD. What else is methylphenidate used for (R17)?

Answer 63

* Features: inappropriate inattention, impulsivity and hyperactivity for age. * Standard medical treatment is with stimulant drugs like methylphenidate and dextroamphetamine as well as parent participation in treatment program and behavioural modifications like goal setting, incentives and punishment * Methylphenidate is a dopaminergic agonist and inhibitor of dopamine reuptake and has been used to treat the behavioural traits associated with medial frontal syndrome. (seen after ischaemia in the distribution of ACA)

Question 64

Witzelsucht: whats the syndrome?

Answer 64

* Witzelsucht is a set of neurological symptoms characterized by the patients uncontrollable tendency to pun, tell inappropriate jokes and pointless or irrelevant stories at inappropriate times. * Patient usually finds these utterances particularly amusing. * Associated with a small lesions of the orbitofrontal cortex.

Question 65

Acute stress reaction vs PTSD

Answer 65

* Acute stress reaction – traumatic event with re-experience of this within 4 weeks of event - present for 2 days to 4 weeks. Must have dissociation * PTSD – similar symptoms, symptoms must be present for more than one month. Ususally happens within 6 months of the event. Delayed PTSD is when it happens later.

Question 66

Torette’s

Answer 66

* History for Tics for over a year (otherwise it’s a transient tic disorder) * Motor tics and verbal tics * Onset must be before the age of 18 •

Question 67

Lithium SE

Answer 67

•Dyspepsia, nausea, vomiting, diarrhoea, hair loss, acne, tremor, decreased cognition, incoordination

Question 68

Kluver-Bucy Syndrome

Answer 68

* Docile affect, excessive abnormal eating, hypersexual behavuour * Bilateral anterior temporal lobe lesions * Can be seen in herpes simplex encephalitis, Picks anoxic-ischaemic lesions in the anterior medial temporal lobes, and after bilateral temporal lobectomy.

Question 69

Huntington’s Disease suicide rate

Answer 69

•Rate of suicide is 13%

Question 70

Balint’s

Answer 70

* optic ataxia, ocular apraxia, and simultanagnosia * Optic ataxia is incoordination of the hand and eye movement – cant pick up items * Ocular apraxia – the inability to voluntarily guide eye movements to a new location of visual fixation and * simultanagnosia – the inability to perceive more than one object at a time even when the objects are in the same place * Caused by bilateral posterior parietal lobe damage • •

Question 71

FTD with ALS - genetics

Answer 71

* Frontotemporal dementias are heterogeneous with the two most common protein accumulations consisting of tau or TDP-43. * Nearly all of the cases of FTD with motor neuron disease have TAR DNA-binding protein 43 (TDP-43) protein accumulations.

Question 72

PSP

Answer 72

•Atypical parkinsonism, eyelid opening apraxia, retrocollis, subcortical dementia including slowness of mental processing and memory retrieval deficit, depression, and supranuclear vertical gaze palsy are all features of progressive supranuclear palsy. • •Global aphasia is not a characteristic clinical feature of progressive supranuclear palsy. •

Question 73

Charles Bonnet Syndrome

Answer 73

•Charles Bonnet syndrome involves visual hallucinations without other psychopathology. It is seen in the elderly and is due to visual loss, with the most common pathology being macular degeneration. The other clinical exam findings are not usually identified with Charles Bonnet syndrome. •

Question 74

Wernicke’s and Korsakoff’s

Answer 74

* Korsakoff amnestic syndrome causes * Amnestic disorder with deficits limited to memory and learning * Language, calculation, visuospatial are spared, digit span should be normal as should motor memory and semantic memory. Only declarative learning and memory are usually involved • • * WERNICKES * confusion, extraocular motility deficits, nystagmus, and ataxia. * Considering this patient is homeless it is likely that he was malnourished resulting in nutritional deficiencies with or without alcoholism. * The administration of glucose without co-administration of thiamine is a precipitant of the acute syndrome. * Give high dose thiamine • •

Question 75

TBI – most common neurobehaviour feature?

Answer 75

•As a general rule the most common neurobehavioral features seen in patients with mild traumatic brain injury and post- concussion syndrome are difficulty with attention, memory impairment, and executive dysfunctio •

Question 76

Intoxication - tell tale signs in alcohol, caffeine, PCP, bath salts, opioids

Answer 76

* Alcohol: slurred speech, Incoordination, unsteady gait, and nystagmus. * Caffeine: restlessness, nervousness, diuresis, GI disturbance, and muscle twitching. * PCP: nystagmus, hypertension, tachycardia, muscle rigidity, dysarthria, decreased responsiveness to pain, seizures. * Bath salts or methylenedioxypyrovalerone (MDPV): hypertension, tachycardia, delusions, hallucinations, and violent behavior. * Opioid intoxication is the only choice that is associated with constricted pupils. The other choices are associated with dilated pupils. • •

Question 77

Antidepressant MOA - citalopram, mirtazapine, bupropion, venlafaxine

Answer 77

* Citalopram: is the most pure selective serotonin reuptake inhibitor (SSRI). * Mirtazapine has a tetracyclic chemical structure and is classified as a noradrenergic and specific serotonergic antidepressant (NaSSA). * Bupropion acts as a norepinephrine reuptake inhibitor and dopamine reuptake inhibitor. * Venlafaxine is an antidepressant of the serotonin-norepinephrine reuptake inhibitor (SNRI) class. Venlafaxine is classified as a noradrenergic and serotonergic antidepressant (NaSSA). •

Question 78

Capgras

Answer 78

•Capgras delusions and psychosis in general are typically thought to originate in the temporal lobe. People who experience this syndrome will have an irrational belief that someone they know or recognize has been replaced by an imposter.

Question 79

Marchiafava-Bignami Disease

Answer 79

•Marchiafava-Bignami disease is seen most often in alcoholics and results in corpus callosum damage. It may present with sudden onset of stupor or coma and seizures. Other patients have acute, subacute, or chronic onset of dementia, gait problems, psychiatric disturbances, incontinence, hemiparesis, aphasia, and apraxia of the left hand (due to callosal disconnection). •

Question 80

Radiation and Chemotherapy - neurological SE

Answer 80

* Both radiation and chemotherapy may also lead to a delayed demyelination of the CNS occurring from 3 months to 5 years after the treatment. * The neurological complications of cranial irradiation include memory loss, cognitive decline, abulia, gait disturbance, ataxia, long tract signs, and tremors. * Patients may become severely disabled with dementia and paresis. * Even prophylatic cranial irradiation has been associated with cases of dementia. * Dementia conditions due to severe demyelination predominately affect the white matter and not the cortex. Alexia with or without agraphia, receptive aphasia, and prosopagnosia all arise due to damage in cortical areas. Memory loss can be a retrieval type or an amnestic type. Amnestic memory loss typically involves the hippocampus or Papez circuit and does not typically arise from white matter abnormalities. Retrieval memory loss is a type of forgetfulness with dramatically improved memory abilities when clues are provided. This type of memory loss typically results from white matter disease.

Question 81

Orbitofrontal cortex lesions - what do you get?

Answer 81

•Lesions in the orbitofrontal cortex may cause personality changes. Symptoms of orbitofrontal lesions may include traits of obsessive compulsive disorder (OCD), disinhibition, hypersexuality, anxiety, depression, impulsiveness, and antisocial behavior. •

Question 82

Familial FTD - inheritance? ## Footnote •

Answer 82

•In familial frontotemporal dementia (FTD), the most common linkage is to chromosome 17q21, although chromosomes 3 and 9 have also been implicated in autosomal dominant inheritance of this condition. Most sporadic cases of frontotemporal dementia have not been linked to specific chromosomal sites. The remaining chromosomes listed in this question have been implicated in familial Alzheimer’s disease.

Question 83

Bilateral globus pallidus interna lesions - what would you expect

Answer 83

•Bilateral globus pallidus interna lesions can cause akinetic mutism. In akinetic mutism, the patient generally has preserved awareness with open eyes, but remains immobile, mute, and does not respond to commands. The globus pallidus interna is part of the anterior cingulate–frontal–subcortical circuit. Recall that bilateral ACA infarcts to the frontal lobes and other lesions to the medial frontal lobes are other causes of akinetic mutism. •

Question 84

Huntington’s Disease genetics

Answer 84

* Huntington’s disease is an autosomal dominant trinucleotide repeat disorder resulting from expansion of CAG repeats on chromosome 4p in a region that codes for the Huntington’s protein. The disease is associated with choreoathetosis and dementia. Chromosome 17 * 989 * has been linked to frontotemporal dementia. Chromosomes 14, 19, and 21 are linked to some familial forms of Alzheimer’s disease. •

Question 85

Don’t forget NPH

Answer 85

•This patient demonstrates the classic triad of normal pressure hydrocephalus (NPH): cognitive dysfunction, gait impairment (often termed “magnetic gait”), and urinary incontinence. MRI brain would be the first best diagnostic test to start with, and reveals ventriculomegaly, which is out of proportion to cortical atrophy •

Question 86

Foix – Chavany Marie syndrome

Answer 86

•Foix–Chavany–Marie syndrome, also known as anterior opercular syndrome, is characterized by severe dysarthria, bilateral voluntary paralysis of the lower cranial nerves with preserved involuntary and emotional innervation. This syndrome is associated with bilateral anterior opercular lesions, frequently in the setting of multiple infarcts. •

Question 87

Apraxias - list them and area of brain from

Answer 87

* Dressing apraxia – non dominant right parietal lobe * Conceptual apraxia is characterized by misconception of the function of objects in the environment - diffuse neurodegenerative processes, as well as with lesions in the nondominant hemisphere. * Ideomotor apraxia - use of a body part as an object during pantomime - dominant (left) parietal cortex, in or around the area of the superior marginal and angular gyrus. * Conduction apraxia - impairment in imitation of movements. The localization of conduction apraxia is not well defined. * Ideational apraxia - impairment in the sequence of motions needed to carry out a specific movement. bifrontal or biparietal dysfunction, as occurs in neurodegenerative disorders. * Disassociation apraxia - inability to execute a movement on command, but with normal ability to imitate. It has most commonly been reported to occur in the left hand in left hemispheric language dominant patients who have left MCA territory lesions. •

Question 88

Frontal Areas - areas and their deficits

Answer 88

* Orbitofrontal: judgement, inhibition of socially inappropriate behaviour * Dorsomedial prefrontal cortex: motor initiation, goal orientated behavoior lesions here or Bilateral Anterior cingulate lesions – apathy, or if severe, akinetic mutism * Dorsolateral prefrontal cortex- planning of motor activity, problem solving, multitasking

Question 89

Frontal eye fields

Answer 89

* The frontal eye fields are located in the middle frontal gyrus, and each frontal eye field is responsible for conjugate eye movement to the contralateral side. A lesion to the right frontal eye fields causes unopposed activity of the left frontal eye fields, leading to eye deviation to the right. A left hemiparesis with conjugate eye deviation to the right suggests a lesion in the right MCA territory. Focal motor seizures that involve the frontal eye fields lead to contralateral convulsions with conjugate gaze deviation to the side of the convulsions (contralateral to the seizure focus). The parapontine reticular formation leads to ipsilateral conjugate gaze, and a lesion in the right pons would lead to a left hemiparesis and left gaze deviation, due to unopposed action of the left parapontine * 1013 * reticular formation. A lesion in the left pons would lead to right hemiparesis with gaze deviation to the right. Gaze deviation with pontine lesions is not overcome by oculocephalic maneuver. •

Question 90

Dejerine-Roussy Syndrome

Answer 90

•Dejerine– Roussy syndrome, or thalamic pain syndrome, which is characterized by initial contralateral hemianesthesia followed weeks later by pain, hyperesthesia, and allodynia. A similar delayed central pain syndrome can occur with a lesion to the medial lemniscus, dorsal columns, or with lesions to the parietal operculum (the latter is also termed pseudothalamic syndrome). The spinothalamic tract projects to the ventral posterolateral nucleus of the thalamus, which in turn projects to the secondary somatosensory cortex (area SII). A lesion to the primary somatosensory cortex (area SI) would lead to contralateral loss of sensation to touch, joint position sense, and vibration, but would spare pain and temperature sensation, and would not cause a delayed pain syndrome. •

Question 91

chromosomes and matched gene = AD

Answer 91

Question 92

pathology - disease match up

Answer 92