Spiegel/BTB Neuro

Question 1

Triad of symptoms is gait ataxia, areflexia, and ophthalmoplegia

Answer 1

Miller–Fisher syndrome is a variant of Guillain–Barré syndrome (acute inflammatory demyelinating polyneuropathy).

Question 2

What causes “drop attacks”

Answer 2

Colloid cyst of third ventricle – “button nose” in middle of ventricular system → appear bright on MRI and filled with cholesterol and protein

Question 3

Diagnosis of CJD

Answer 3

!) CSF assay for 14-3-3 proteinase inhibitor
2) (best)test is Rt-QulC that looks for abnormal prion proteins in CSF

Question 4

Persistent vegetative state symptoms

Answer 4

ABSENT cognitive function/awareness of the surrounding environment
PRESENT: sleep–wake cycle; Spontaneous movements; eye opening/tracking; swallowing

Question 5

Persistent vegetative state symptoms timeframe

Answer 5

Needs to have at least month in coma
Permanent irreversible brain damage is believed to set in after about 12 months of a persistent vegetative state that follows brain trauma and usually about 3 or more months following anoxic brain injury.

Question 6

Wernicke’s encephalopathy autopsy finding:

Answer 6

microhemorrhages in the periventricular gray matter - especially aqueduct + 3rd and 4th ventricles

Question 7

conduction vs Broca’s vs Wernicke’s aphasia (symptoms)

Answer 7

Conduction aphasia → inability to repeat, relatively normal spontaneous speech, and the possibility of paraphasic errors and hesitancy

Broca’s aphasia → broken, stuttering, staccato speech, with inability to repeat and phonemic and paraphasic error

Wernicke’s aphasia → speech is generally fluent, but comprehension is impaired. Speech may also be logorrheic, or over productive. The speech displays paragrammatism, which involves neologisms, verbal paraphasic errors, and production of jargon. Repetition, naming, and auditory comprehension are impaired. Reading comprehension is impaired.

Question 8

Conduction aphasia location

Answer 8

Dominant (left) inferior parietal or superior temporal regions
–> Associated symptoms: right hemiparesis, right hemisensory loss, right hemianopsia, and limb apraxia

Question 9

Broca’s area

Answer 9

in the left posterior inferior frontal gyrus

Question 10

Wernicke’s area

Answer 10

lesion in the superior temporal gyrus

Question 11

EEG: periodic sharp waves

Answer 11

CJD

Question 12

EEG: 3 Hz/ 3 per second spike and wave

Answer 12

Absence seizure

Question 13

EEG: Periodic lateralizing epileptiform discharges (PLEDs)

Answer 13

HSV encephalitis, abscess, hemispheric stroke, meningitis, or tumors

Question 14

EEG: abnormal interictal high amplitude waves and a background of irregular spikes. There is continuous (during wakefulness), high-amplitude (>200 Hz), generalized polymorphic slowing with no organized background and multifocal spikes

Answer 14

infantile spasms → hypsarrhythmia

Question 15

Degeneration of spinal cord in ALs

Answer 15

anterior horn degeneration

Question 16

Tuberous sclerosis

Answer 16

Autosomal dominant seizures

• IDD
• Ash leaf spot
• hamartomas
• cutaneous lesions,
• shagreen spots (no eye lesions ever)

Question 17

Syndrome: Cafe-au-lait, neurofibromas, schwannomas is due to _____ gene on chromosome __

Answer 17

neurofibromatosis type 1; NF1 gene on chromosome 17

Question 18

Autosomal Dom syndrome caused by microdeletion of chromosome 7q11

Answer 18

Williams Syndrome

Question 19

Gerstmann’s syndrome

Answer 19

left parietal lobe stroke (left angular gyrus) ⇒

• Acalculia
• Agraphia (without alexia)
• right and left confusion
• finger Agnosia

Question 20

Reflex and Spinal Cord Level:

Ankle

Patellar

Biceps

Nipple

Umbilicus

front leg, foot flexion ; lateral leg

*

Answer 20

• T4 → nipple
• T10 → umbilicus
• C5 → biceps reflex
• L5 → front leg, foot flexion ; lateral leg
• L4 → patellar
• S1 → ankle

Question 21

chromosome 19 indicated in what type of headache

Answer 21

Migraine

Question 22

Syndrome d/t chromosome 15 deletion that is paternally pass along

Answer 22

Prader-Willi

• hypotonic baby
• IDD
• ASD
• Aggressive/temper tantrums
• Overeating

Question 23

MOST COMMON cause of CN III palsy

Answer 23

Diabetes;

Question 24

Carbon monoxide poisoning leads to lesion in this area of the brain

Answer 24

b/l globus pallidus necrosis (Also MDMA, cocaine, cyanide, opiates)

Question 25

* ipsilateral ptosis, miosis, facial anhidrosis

Answer 25

Horner’ Syndrome

Question 26

1. Ab to this enzyme result in Stiff person syndrome? 2. Symptoms?

Answer 26

1. glutamic acid decarboxylase (anti-GAD) 2. paraneoplastic (breast, lung, hodgkins) syndrome ⇒

Question 27

contralateral hemiplegia, conjugate eye deviation (to side of infarct), hemianesthesia,

Answer 27

MCA stem occlusion

Question 28

Brown-Sequard

Answer 28

* spinal hemisection ⇒ * Loss of motor + mvp IPSILATERAL to and below the level of dissection * Loss of pain + and temp CONTRALATERAL to 1-2 dermatomes below dissection

Question 29

Alexia **without** agraphia

Answer 29

* Can write, but cant’ read their own writing * LEFT PCA Stroke * splenium of corpus callosum

Question 30

**Progressive Supranuclear Palsy**

Answer 30

* vertical gaze palsy ; pseudobulbar/corticobulbar; frontal lobe signs * Levodopa only helps at start/temporarily in treatment

Question 31

_Sleepwalking in children occurs during which stage of sleep?_

Answer 31

_Stage IV sleep_

Question 32

* Neurological location for taste

Answer 32

Insular cortex / sylvian

Question 33

**MELAS SYNDROME**

Answer 33

**Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes** * most common _mitochondrial_ disease * muscle bx with **red ragged fibers** * Cerebral strokes *

Question 34

Where does **_B_**otulinum toxin act?

Answer 34

**_bl_**_ocks_ **_ac_**_etylcholine release at presynaptic nerve terminal_

Question 35

small, irregular pupils that constrict to accommodation, but not to light.

Answer 35

**Argyll Robertson pupils** * Tabes dorsalis is the spinal form of syphilis and develops about 15 to 20 years after the initial infection *

Question 36

**Klüver–Bucy syndrome**

Answer 36

* bilateral destruction of the amygdaloid bodies and the inferior temporal cortex ⇓ * hypersexuality, hyperphagia, and hyperorality

Question 37

**Balint’s Syndrome**

Answer 37

* Ischemia/lesion bilateral parietal/occipital lobes or occipital lobes → vascular dementia * → “top of the basilar” syndrome → delirium, peduncular hallucinosis, obtundation, impaired memory * skewed eyes → ocular apraxia, optic ataxia, visual attention * w/ Simultagnosia → inability to perceive a scene

Question 38

peduncular hallucinosis

Answer 38

Vivid, colorful visions of people and animals

Question 39

**Meige Syndrome & Treatment**

Answer 39

blepharospasm and oromandibular dystonia; Treat w/ botulinum toxin type A

Question 40

Syringomyelia findings

Answer 40

* bilateral loss of pain + temp @ level of lesion⇒muscle weakness ⇒ “cape-like” flaccid paralyisis & atropy of upper limb 2/2 ventral horn cell destruction; horner's syndrome

Question 41

Prion disease

Answer 41

Question 42

What type of neuropathy seen in inhalant abuse?

Answer 42

Distal sensory

Question 43

**Devic’s disease**

Answer 43

**AKA: neuromyelitis optica** * disease of demyelination → presents with cervical myelopathy + bilateral optic neuropath

Question 44

most common neuropathy associated with HIV

Answer 44

Distal symmetric axonal polyneuropathy

Question 45

In which psychiatric condition do you see abnormal hyperintensities in subcortical regions: periventricular regions, basal ganglia, and thalamus

Answer 45

MDD

Question 46

In which psychiatric condition do you see abnormal hyperintensities in subcortical regions: periventricular regions, basal ganglia, and thalamus

Answer 46

MDD

Question 47

* _________ lesion: profanity, irritable, disinhibited * _________ lesion: apathy * _________ lesion: depression * _________ lesion: mania , euphoria

Answer 47

* **Orbitofrontal** lesion: profanity, irritable, disinhibited * Apathy: **dorsolateral**, **internal capsule** * **Left frontal** lesion: depression * **Right frontal** lesion: mania , euphoria

Question 48

Tay- Sachs Enzyme dfcy and inheritance patter

Answer 48

* hexosaminidase A def → aut recessive

Question 49

Declarative vs. Episodic memory

Answer 49

* Declarative memory → recall of facts * Episodic memory → medial temporal lobes/anterior thalamic nuclei → declarative awareness (what you ate that day)

Question 50

**Semantic memory vs. Procedural memory**

Answer 50

* Semantic memory → Inferolateral temporal lobes → declarative awareness (who first president was) * Procedural memory → basal ganglia/cerebellum → non-declarative ; driving a vehicle (explicit) or typing your telephone number (implicit)

Question 51

Krabbe’s disease

Answer 51

* AKA (globoid leukodystrophy) → deficient galactocerebrosidase B-galactosidase → aut recessive

Question 52

Lesch-Nyhan

Answer 52

* Lesch-Nyhan → hypoxanthine-guanine phosphoribosyltransferase → hyperuricemia, * **x-linked** recessive

Question 53

Niemann-Pick disease

Answer 53

* IN CHILDHOOD * sphingomyelinase deficiency ⇒lysosomal storage disease * SMPD1 gene * hepatosplenomegaly + cherry red macula + areflexia * **autosomal recessive inheritance**

Question 54

[_Nucleus Basalis of Meynert_](https://www.sciencedirect.com/topics/neuroscience/nucleus-basalis-of-meynert)

Answer 54

a large population of **cholinergic neurons** that project their axons to the entire cortical mantle, the olfactory tubercle, and the amygdala. It has been functionally associated with the **control of attention and maintenance of arousal**

Question 55

Lewy bodies are composed of which substances?

Answer 55

alpha synuclein, neurofilament, and ubiquitin

Question 56

Role of alpha synuclien in Lewy Body dementia?

Answer 56

* Alpha-synuclein plays a role in vesicle transportation and plays a role modulating DNA repair processes. * compromised ⇒ cell death * fluctuation cognition * visual hallucinations * Parkinsonism sxs only after 1 year of cognitive decline * neuroleptic sensitivity * REM sleep behavior disorder

Question 57

Which part of the hypothalamus is involved in the switch between wakefulness and sleep?

Answer 57

ventrolateral preoptic nucleus (VLPO or VLPN) * *Neurons in this area help to promote sleep by inhibiting activity in areas of the brainstem that maintain wakefulness.*

Question 58

What is the most common cause of pellagra?

Answer 58

* **Vitamin B3, or niacin, deficiency→** diarrhea, dermatitis, and dementia * Photosensitivity, glossitis, ataxia, and dilated cardiomyopathy ⇒ Left untreated, death from the disease will occur within 4 or 5 years

Question 59

Pseudobulbar Affect

Answer 59

* present in 50% of individuals with ALS. * It presents as sudden pathological crying or laughing.

Question 60

Manifestations of Zinc deficiency

Answer 60

* cognitive dysfunction in children * diarrhea * skin lesions/hair loss * increased incidence of pneumonia.

Question 61

**Kleine-Levin syndrome**

Answer 61

Hypersomnia, aggressive, and over sexual behavior. Tx: modafinil helps with sleep-wake disorders

Question 62

Brain changes in PTSD

Answer 62

* _reduced hippocampal volume_ * reduced MLPFC activation * reduced frontal cortex volume – dysregulation of executive function * reduced ACC → extinction of condition fear impaired * *increase amygdala activity →* involved in mediating stress response, emotional processing, and acquisition of fear responses

Question 63

What changes due you see in the brains of people with autism spectrum disorder?

Answer 63

cerebellum and limbic system is abnormally structured. T_here is a reduction in the number or atrophy of Purkinje cells in the cerebellum_

Question 64

Which tract that connects Broca's area and Wernicke's area?

Answer 64

**Arcuate fasciculus** forms a tract that connects Broca's area (located in the left frontal region) and Wernicke's area located in the lef temporal region. It is associated with producing and understanding language and is associated with AH in patients with schizophrenia.

Question 65

Which area of the brain is implicated in dyslexia?

Answer 65

**planum temporale**, a triangular area part of the superior temporal cortex located in the Wernicke area * involved in language, auditory processing, as well as sustaining phonological representations * Dyslexic Pts found to have a greater rightward asymmetry ⇒less developed neural network for reading

Question 66

Symptoms seen in OVERactive caudate vs UNDERactive caudate?

Answer 66

* Overacting caudate →D2 overstimulation → tics and extraneous motor movements * Under acting caudate → D2 inhibition→ bradykinesia

Question 67

Lead poisoning

Answer 67

* **encephalopathy and anemia** * neuropathy W/_motor dysfunction_ * *symmetric distal* weakness associated * atrophy, reflex loss, and occasionally fasciculations

Question 68

aluminum poisining

Answer 68

* **neurocognitive disorder (NCD)** * fractures (ostemalacia) * anemia (micro d/t impaired Fe absorption)

Question 69

Manganese toxicity

Answer 69

parkinsonism-like symptoms and "manganese madness" (emotional lability, confusion, somnolence, hallucinations, and compulsive and impulsive acts).

Question 70

Bismuth toxicity

Answer 70

* blue-black gum line, * lichen planus-like skin lesions * encephalopathy with confusion and seizure * GI symptoms/black stools.

Question 71

Cyanide poisoning

Answer 71

* generalized weakness, confusion, bizarre behavior, excessive sleepiness, coma, shortness of breath, headache, dizziness, and seizures.

Question 72

**ANTEROGRADE amnesia location vs RETROGRADE amnesia**

Answer 72

* **ANTEROGRADE amnesia → Hippocampus,** ***"a hippo is bigger than ant"*** * **RETROGRADE amnesia → Thalamus**

Question 73

**Poor prognosticators of cerebral anoxia**

Answer 73

* Duration of anoxia longer than 8 to 10 minutes * duration of CPR longer than 30 minutes * duration of postanoxic coma longer than 72 hours * pupillary light reaction absent on day 3 * motor response to pain absent on day 3 * Glasgow coma scale on day 3 less than 5 * brain stem reflexes absenr * blood glucose on admission of greater than 300 mg/dl.

Question 74

4 types of diabetic neuropathy

Answer 74

1. **_Peripheral_** neuropathy is the most common type of diabetic neuropathy and causes pain or loss of feeling in the toes, feet, legs, hands, and arms. 2. **_Autonomic_** neuropathy causes alterations in digestion, bowel and bladder function, sexual response, and perspiration. It may also affect the nerves that serve the heart and monitor blood pressure, as well as nerves in the eyes and lungs, and it can cause hypoglycemia unawareness, in which patients no longer experience warning symptoms associated with low blood sugar. 3. **_Proximal_** neuropathy tends to cause pain in the thighs, hips, or buttocks, and can lead to weakness in the legs. 4. **_Focal_** neuropathy results in the sudden weakness of 1 nerve or a group of nerves, causing muscle weakness or pain. Any nerve in the body can be affected.

Question 75

wAVE ACTIVITY IN STAGES OF SLEEP

Answer 75

* *Alpha activity** is seen when the patient is a**wake but has their eyes closed and during N1 sleep.** There will be associated low voltage mixed frequency activity during wakefulness. * *Theta activity** is seen when the patient is **under late N1 and throughout N2** stages of NREM sleep. **K complexes with background sleep bundles** and low voltage mixed frequency activity are seen in patients under stage **N2 NREM** sleep. * *High amplitude delta (slow**) waves are seen in patients under stage **N3 NREM sleep.** * *Sawtooth waves** are seen in patients under **(REM)** rapid eye movement sleep.

Question 76

Left medial medullary syndrome

Answer 76

* AKA ***inferior alternating syndrome*** * anterior spinal artery * **ipsilateral** paresis, atrophy, and fibrillations of the _tongue_ * **contralateral** _hemiplegia sparing the face_ from pyramidal tract involvement, * **contralateral** loss of _position and vibratory_ sensation from involvement of the medial leminiscus, * occasionally upbeat nystagmus if the extent of the infarct extends dorsally towards the medial longitudinal fasciculus.

Question 77

Located in the dorsal midbrain and consists of the superior colliculus (visual reflexes) and inferior colliculi (auditory reflexes)

Answer 77

* Tectum

Question 78

Imaging finding in patient with CJD?

Answer 78

Multifocal gray matter hyperintensities in subcortical and cortical regions on neuroimaging

Question 79

Whipple's disease

Answer 79

* caused by the bacterium Tropheryma whipplei * creates a malabsorption syndrome * skin rashes, diarrhea, arthritis, and cognitive decline * It can be successfully treated with a long course of antibiotics, such as doxycycline and hydroxychloroquine.