Spiegel/BTB Neuro Flashcards
Triad of symptoms is gait ataxia, areflexia, and ophthalmoplegia
Miller–Fisher syndrome is a variant of Guillain–Barré syndrome (acute inflammatory demyelinating polyneuropathy).
What causes “drop attacks”
Colloid cyst of third ventricle – “button nose” in middle of ventricular system → appear bright on MRI and filled with cholesterol and protein
Diagnosis of CJD
!) CSF assay for 14-3-3 proteinase inhibitor
2) (best)test is Rt-QulC that looks for abnormal prion proteins in CSF
Persistent vegetative state symptoms
ABSENT cognitive function/awareness of the surrounding environment
PRESENT: sleep–wake cycle; Spontaneous movements; eye opening/tracking; swallowing
Persistent vegetative state symptoms timeframe
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.
Wernicke’s encephalopathy autopsy finding:
microhemorrhages in the periventricular gray matter - especially aqueduct + 3rd and 4th ventricles
conduction vs Broca’s vs Wernicke’s aphasia (symptoms)
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.
Conduction aphasia location
Dominant (left) inferior parietal or superior temporal regions
–> Associated symptoms: right hemiparesis, right hemisensory loss, right hemianopsia, and limb apraxia
Broca’s area
in the left posterior inferior frontal gyrus
Wernicke’s area
lesion in the superior temporal gyrus
EEG: periodic sharp waves
CJD
EEG: 3 Hz/ 3 per second spike and wave
Absence seizure
EEG: Periodic lateralizing epileptiform discharges (PLEDs)
HSV encephalitis, abscess, hemispheric stroke, meningitis, or tumors
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
infantile spasms → hypsarrhythmia
Degeneration of spinal cord in ALs
anterior horn degeneration
Tuberous sclerosis
Autosomal dominant seizures
- IDD
- Ash leaf spot
- hamartomas
- cutaneous lesions,
- shagreen spots (no eye lesions ever)
Syndrome: Cafe-au-lait, neurofibromas, schwannomas is due to _____ gene on chromosome __
neurofibromatosis type 1; NF1 gene on chromosome 17
Autosomal Dom syndrome caused by microdeletion of chromosome 7q11
Williams Syndrome
Gerstmann’s syndrome
left parietal lobe stroke (left angular gyrus) ⇒
- Acalculia
- Agraphia (without alexia)
- right and left confusion
- finger Agnosia
Reflex and Spinal Cord Level:
Ankle
Patellar
Biceps
Nipple
Umbilicus
front leg, foot flexion ; lateral leg
*
- T4 → nipple
- T10 → umbilicus
- C5 → biceps reflex
- L5 → front leg, foot flexion ; lateral leg
- L4 → patellar
- S1 → ankle
chromosome 19 indicated in what type of headache
Migraine
Syndrome d/t chromosome 15 deletion that is paternally pass along
Prader-Willi
- hypotonic baby
- IDD
- ASD
- Aggressive/temper tantrums
- Overeating
MOST COMMON cause of CN III palsy
Diabetes;
Carbon monoxide poisoning leads to lesion in this area of the brain
b/l globus pallidus necrosis (Also MDMA, cocaine, cyanide, opiates)
- ipsilateral ptosis, miosis, facial anhidrosis
Horner’ Syndrome
- Ab to this enzyme result in Stiff person syndrome?
- Symptoms?
- glutamic acid decarboxylase (anti-GAD)
- paraneoplastic (breast, lung, hodgkins) syndrome ⇒
contralateral hemiplegia, conjugate eye deviation (to side of infarct), hemianesthesia,
MCA stem occlusion
Brown-Sequard
- 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
Alexia without agraphia
- Can write, but cant’ read their own writing
- LEFT PCA Stroke
- splenium of corpus callosum
Progressive Supranuclear Palsy
- vertical gaze palsy ; pseudobulbar/corticobulbar; frontal lobe signs
- Levodopa only helps at start/temporarily in treatment
Sleepwalking in children occurs during which stage of sleep?
Stage IV sleep
- Neurological location for taste
Insular cortex / sylvian
MELAS SYNDROME
Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes
- most common mitochondrial disease
- muscle bx with red ragged fibers
- Cerebral strokes
*
Where does Botulinum toxin act?
blocks acetylcholine release at presynaptic nerve terminal
small, irregular pupils that constrict to accommodation, but not to light.
Argyll Robertson pupils
- Tabes dorsalis is the spinal form of syphilis and develops about 15 to 20 years after the initial infection
*
Klüver–Bucy syndrome
- bilateral destruction of the amygdaloid bodies and the inferior temporal cortex ⇓
- hypersexuality, hyperphagia, and hyperorality
Balint’s Syndrome
- 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
- skewed eyes → ocular apraxia, optic ataxia, visual attention
peduncular hallucinosis
Vivid, colorful visions of people and animals
Meige Syndrome & Treatment
blepharospasm and oromandibular dystonia;
Treat w/ botulinum toxin type A
Syringomyelia findings
- 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
Prion disease
What type of neuropathy seen in inhalant abuse?
Distal sensory
Devic’s disease
AKA: neuromyelitis optica
- disease of demyelination → presents with cervical myelopathy + bilateral optic neuropath
most common neuropathy associated with HIV
Distal symmetric axonal polyneuropathy
In which psychiatric condition do you see abnormal hyperintensities in subcortical regions: periventricular regions, basal ganglia, and thalamus
MDD
In which psychiatric condition do you see abnormal hyperintensities in subcortical regions: periventricular regions, basal ganglia, and thalamus
MDD
- _________ lesion: profanity, irritable, disinhibited
- _________ lesion: apathy
- _________ lesion: depression
- _________ lesion: mania , euphoria
- Orbitofrontal lesion: profanity, irritable, disinhibited
- Apathy: dorsolateral, internal capsule
- Left frontal lesion: depression
- Right frontal lesion: mania , euphoria
Tay- Sachs Enzyme dfcy and inheritance patter
- hexosaminidase A def → aut recessive
Declarative vs. Episodic memory
- Declarative memory → recall of facts
- Episodic memory → medial temporal lobes/anterior thalamic nuclei → declarative awareness (what you ate that day)
Semantic memory vs. Procedural memory
- 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)
Krabbe’s disease
- AKA (globoid leukodystrophy) → deficient galactocerebrosidase B-galactosidase → aut recessive
Lesch-Nyhan
- Lesch-Nyhan → hypoxanthine-guanine phosphoribosyltransferase → hyperuricemia,
- x-linked recessive
Niemann-Pick disease
- IN CHILDHOOD
- sphingomyelinase deficiency ⇒lysosomal storage disease
- SMPD1 gene
- hepatosplenomegaly + cherry red macula + areflexia
- autosomal recessive inheritance
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
Lewy bodies are composed of which substances?
alpha synuclein, neurofilament, and ubiquitin
Role of alpha synuclien in Lewy Body dementia?
- 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
Which part of the hypothalamus is involved in the switch between wakefulness and sleep?
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.
What is the most common cause of pellagra?
- 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
Pseudobulbar Affect
- present in 50% of individuals with ALS.
- It presents as sudden pathological crying or laughing.
Manifestations of Zinc deficiency
- cognitive dysfunction in children
- diarrhea
- skin lesions/hair loss
- increased incidence of pneumonia.
Kleine-Levin syndrome
Hypersomnia, aggressive, and over sexual behavior. Tx: modafinil helps with sleep-wake disorders
Brain changes in PTSD
- 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
What changes due you see in the brains of people with autism spectrum disorder?
cerebellum and limbic system is abnormally structured. T_here is a reduction in the number or atrophy of Purkinje cells in the cerebellum_
Which tract that connects Broca’s area and Wernicke’s area?
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.
Which area of the brain is implicated in dyslexia?
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
Symptoms seen in OVERactive caudate vs UNDERactive caudate?
- Overacting caudate →D2 overstimulation → tics and extraneous motor movements
- Under acting caudate → D2 inhibition→ bradykinesia
Lead poisoning
- encephalopathy and anemia
- neuropathy W/motor dysfunction
- symmetric distal weakness associated
- atrophy, reflex loss, and occasionally fasciculations
aluminum poisining
- neurocognitive disorder (NCD)
- fractures (ostemalacia)
- anemia (micro d/t impaired Fe absorption)
Manganese toxicity
parkinsonism-like symptoms and “manganese madness” (emotional lability, confusion, somnolence, hallucinations, and compulsive and impulsive acts).
Bismuth toxicity
- blue-black gum line,
- lichen planus-like skin lesions
- encephalopathy with confusion and seizure
- GI symptoms/black stools.
Cyanide poisoning
- generalized weakness, confusion, bizarre behavior, excessive sleepiness, coma, shortness of breath, headache, dizziness, and seizures.
ANTEROGRADE amnesia location vs RETROGRADE amnesia
- ANTEROGRADE amnesia → Hippocampus, “a hippo is bigger than ant”
- RETROGRADE amnesia → Thalamus
Poor prognosticators of cerebral anoxia
- 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.
4 types of diabetic neuropathy
- 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.
- 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.
- Proximal neuropathy tends to cause pain in the thighs, hips, or buttocks, and can lead to weakness in the legs.
- 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.
wAVE ACTIVITY IN STAGES OF SLEEP
- *Alpha activity** is seen when the patient is awake 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.
Left medial medullary syndrome
- 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.
Located in the dorsal midbrain and consists of the superior colliculus (visual reflexes) and inferior colliculi (auditory reflexes)
- Tectum
Imaging finding in patient with CJD?
Multifocal gray matter hyperintensities in subcortical and cortical regions on neuroimaging
Whipple’s disease
- 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.
