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FA complete review part 3.2 Pathology

What is Aphasia? Higher-order language deficit
Inability to understand/produce/use language appropriately. Aphasia
What is the cause for Aphasias? Pathology in dominant cerebral hemisphere
A condition that affects the dominant cerebral hemisphere, that causes the patient to inappropriately verbally express. Aphasia
Which is the most commonly affected Dominant cerebral hemisphere in aphasia? Left (usually the dominant side)
What is Dysarthria? Motor inability to speak
Is Aphasia or Dysarthria the inability to produce movement in order to speak? Dysarthria
What are the two criteria tested or analyzed in Aphasias? Speech fluency and Comprehension
What are the main two categories in which aphasias are usually divided into? 1. Repetition impaired 2. Repetition intact
What specific language and speech characteristic is used to categorize the most significant types of aphasias? Repetition ability
List of the Repetition IMPAIRED aphasias: 1. Broca (expressive) 2. Wernicke (receptive) 3. Conduction 4. Global
Which repetition impaired aphasia have NON-fluent speech fluency? Broca and Global
Which is the only repetition impaired aphasia that has both affected, speech fluency and comprehension? Global aphasia
Broca area location Inferior frontal gyrus of frontal lobe
What area/structure is at the inferior frontal gyrus of frontal lobe? Broca area
How is a Broca aphasia patient commonly found (emotionally) during doctor's visits or therapy? Frustrated
A patient with a broken language, goes to doctor's office, but during the visit the patient, loses patience and acts extremely frustrated and agitated. Dx? Broca aphaia
Broca aphae is _______________________. Expressive
Wernicke aphasia is denominated a __________________ aphasia. Receptive
What type of aphasia is most likely indicated if it's described as an "expressive aphasia" with repetition impaired? Broca aphasia
Speech fluency = Nonfluent Comprehension = Intact Repetition = Impaired What is the most likely Dx? Broca aphasia
Speech fluency = Fluent Comprehension = Impaired Repetition = Impaired Wernicke's aphasia
How are the speech fluency and comprehension in a Wernicke's aphasia? Fluent speech but impaired or poor comprehension
Patients do not have insight. Aphasia? Wernicke's aphasia
Superior temporal gyrus of temporal lobe. Wernicke area
How is the "insight" of a Broca aphasia patient? Intact
What kind of aphasia is seen by damage to the Arcuate fasciculus? Conduction aphasia
What is the only deficit seen in Conduction aphasia? Repetition
Speech fluency = Fluent Comprehension = Intact Repetition = Impaired Conduction aphasia
If both areas, Broca and Wernicke areas, are affected, what is the most likely type of repetition impaired aphasia to be developed? Global aphasia
What are the two repetition impaired aphasias that develop due to damage to the Arcuate fasciculus? Conduction and Global aphasias
What is the key word in the name of aphasias that have INTACT repetition? Transcortical
If the aphasia in question has the word "Transcortical" it always depicts what characteristic? Repetition is INTACT
What are the 3 types of Repetition intact aphasias? 1. Transcortical motor 2. Transcortical sensory 3. Transcortical, mixed
Speech fluency = Nonfluent Comprehension = Intact Repetition = Intact Transcortical motor aphasia
Affects frontal love around Broca area, but Broca area is spared. Dx? Transcortical motor aphasia
What area is spared from damage in Transcortical motor aphasia? Broca area
What areas are affected in Transcortical motor aphasia? Frontal lobe around Broca area
Speech fluency = Fluent Comprehension = Impaired Repetition = Intact Transcortical sensory aphasia
Which aphasia affects the temporal lobe around the Wernicke area, but Wernicke area is actually spared (non affected)? Transcortical sensory aphasia
Speech = Nonfluent Comprehension = Impaired Repetition = Intact Transcortical, mixed aphasia
What areas are spared from damage in Transcortical, mixed aphasia? Broca and Wernicke areas and Arcuate fasciculus remain intact.
What is affected in order to develop Transcortical, mixed aphasia? Watershed areas
Watershed areas of brain are affected. What pathology is associated or develope due to this effect? Transcortical, mixed aphasia
When is comprehension affected in aphasias? The Wernicke area or its surroundings are affected
When is Speech fluency affected in aphasias? Broca area or its surroundings are affected/damaged
What is the definition of aneurysms? Abnormal dilation of an artery due to weakening of vessel wall
Abnormal dilation of an artery due to weakening of vessel wall Aneurysm
What are the two main types of aneurysms? 1. Saccular aneurysm 2. Charcot-Bouchard microaneurysm
What is another way to name a Saccular aneurysm? Berry aneurysm
Where do Berry (Saccular) aneurysms occur? Bifurcations of the circle of Willis
Where is the MC site for a Berry aneurysm? Junction of Anterior Communicating artery (ACom) and ACA.
What are two conditions associated with development of Berry aneurysms? ADPKD and Ehlers-Danlos syndrome
What are secondary (minor) associated risk factors of Berry aneurysm development? Advance age, hypertension, smoking, African-American race
Which race is most commonly affected by Berry aneurysms? African-Americans
Pathway in which a Saccular aneurysm develops focal neurological deficits: Rupture of Saccular aneurysm --> Subarachnoid hemorrhage leading to focal neurological deficits
What common ways patients describe the symptoms of Subarachnoid hemorrhage? 1. "worst headache of my life", 2. "thunderclap headache"
The direct compression of the Anterior Communicating artery by a Saccular aneurysm produces the following symptoms: - Bitemporal hemianopia; - Visual acuity deficits - Rupture --> ischemia in ACA distribution leading to contralateral lower extremity hemiparesis, and sensory deficits
What is the result of ACom rupture because of a Berry aneurysm in the ACA? Contralateral lower extremity hemiparesis and sensory deficits
What is the result of MCA distribution obstruction due to a Berry aneurysm ruptures? Contralateral upper extremity and lower facial hemiparesis, and sensory deficits
PCom compression due to direct compression of a Berry aneurysm ---> Ipsilateral CN III palsy leading to mydriasis; and possible ptosis, "down and out" eye.
What are arteries commonly directly compressed by a Saccular aneurysms? 1. ACom 2. MCA 3. PCom
What is a common aneurysm, associated with chronic hypertension? Charcot-Bouchard microaneurysm
What small vessels are commonly affected by Charcot-Bouchard microaneurysm? Lenticulostriate arteries in basal ganglia and Thalamus
What is a common severe complication of Charcot-Bouchard aneurysms? Lacunar strokes
What is a common cause of Lacunar strokes? Charcot-Bouchard microaneurysms
Seizures are characterized by: Synchronized, high-frequency neuronal firing
What are the two main categories of Seizures? 1. Partial (focal) seizures 2. Generalized seizures
What are partial (focal) seizures? Affect single area of the brain. Most commonly originate in medial temporal lobe.
What are the types of Partial (focal) seizures? 1. Simple partial seizures 2. Complex partial seizures
Description of Simple partial seizures? - Consciousness intact - Motor, sensory, autonomic, and psychic
Which type of focal seizure is characterized with impaired consciousness, and automatisms? Complex partial seizures
What conditions (pathologies) are associated with Partial (focal) seizures? 1. Epilepsy 2. Status epilepticus
What is Epilepsy? A disorder of recurrent seizures
What is Status epilepticus? Continuous (> 5 mins) or recurring seizures that may result in brain injury
Condition of recurrent seizures less than 5 minutes, that result in brain injury. Status epilepticus
Diffuse seizures = Generalized seizures
What are the types of Generalized seizures? 1. Absence 2. Myoclonic 3. Tonic-clonic 4. Tonic 5. Atonic
What is another way to name an absence seizure? Petit mal
3 Hz spike-and-wave discharges, no postictal confusion, and blank stare. Absence seizure
What type of generalized seizures is featured by a "blank stare"? Absence seizure
Description of Myoclonic seizures: Quick, repetitive jerks
What is another way to refer to a Tonic-clinic seizure? Grand mal
If a vignette describes a "grand mal" seizure, it refers to: Tonic-clonic seizure
What is the description of and Tonic-clonic seizure? Alternating stiffening and movement
Person fall on floor and has a pattern of body stiffening and movement alternation. Dx? Tonic-clonic seizure
What is the term "tonic" refer in seizures? Stiffening
Description Atonic seizure? "drop" seizures (falls to floor); commonly mistaken for fainting
"drop" seizures Atonic seizures
What is the MCC of elderly seizures? Stokes
What is another way to refer infection seizure? Febrile seizure
What is a headache? Pain due to irritation of structures such as the dura, cranial nerves, or extracranial structures.
What types of headaches are more common in males? Cluster headaches
What are the types of headaches important for USMLE? 1. Cluster 2. Tension 3. Migraine
Which type (s) of headache are unilateral? Cluster and Migraine
Which type of headache is bilateral? Tension
What is the duration of a Cluster headache? 15 min - 3 hour; repetitive
What is the description of a Cluster headache? - Excruciating periorbital pain with lacrimation and rhinorrhea - (+/-) Horner syndrome - More common in males
What is the acute treatment of a Cluster headache? Sumatriptan, 100% oxygen
What is the prophylaxis medication for cluster headache? Verapamil
What is the average duration of a Tension headache? > 30 minutes
Description of Tension headache - Steady, "band-like" pain. - No photophobia or phonophobia - No aura
What are the common drugs used for acute tension headache? Analgesics, NSAIDs, and acetaminophen
What medication is commonly used to treat chronic tension headaches? Amitriptyline
Unilateral, intense 4-72 hour headache. Migraine
What is the duration of a migraine? 4 - 72 hours
What is the description of a Migraine? - Pulsating pain with nausea, photophobia, or phonophobia - (+/-) have "aura"
What is the cause for a migraine? Irritation of CN V, meninges, or blood vessels
What serum substances are secreted in the pathogenesis of a migraine? Substance P, calcitonin gene-related peptide, and vasoactive peptides
What is the acute treatment of a migraine? NSAIDs, triptans, and dihydroergotamine
Prophylaxis of a migraine: Lifestyle changes, B-blockers, amitriptyline, topiramate, and valproate.
What is a common mnemonic of migraine characteristics? POUND Pulsatile One-day duration Unilateral Nausea Disabling
What are less common causes of headache? Subarachnoid hemorrhage, meningitis, hydrocephalus, neoplasia, and giant cell (temporal) arteritis.
A cluster headache is often compared with what other pathology? Trigeminal neuralgia
Producers repetitive, unilateral, shooting pain in the distribution of CN V. Trigeminal neuralgia
What is the first line of therapy for Trigeminal neuralgia? Carbamazepine
What can trigger Trigeminal neuralgia? Chewing, talking, touching certain parts of the face
What is "Akathisia"? Restlessness and intense urge to move
Restlessness and intense urge to move. Akathisia
Possible side effect of Parkinson treatment Akathisia
What is Asterixis? Extension of wrists causes "flapping" motion
What pathologies and/or conditions are associated with Asterix? Hepatic encephalopathy, Wilson disease, and other metabolic derangements.
What are two common movement disorders associated with lesion of the Basal ganglia? Athetosis and Chorea
What is Athetosis? Slow, snake-like, writhing movements; especially in the fingers
Slow, snake-like, writhing movements in the fingers Athetosis
Definition of Chorea: Sudden, jerky, purposeless movements
Sudden, jerky, and purposeless movements Chorea
What conditions are seen with Chorea? Huntington disease and Rheumatic fever
Dystonia is: Sustained, involuntary muscle contractions
An involuntary muscle contraction that is sustained for short period of times. Dystonia
Associated conditions that may produce Dystonia: Writer's cramp, blepharospasm, and torticollis
"Writer's cramp" is an example of what type of movement disorder? Dystonia
What is Essential tremor? High-frequency tremor with sustained posture, worsened with movement or when anxious
High-frequency tremor with sustained posture, that is worst in movements of anxiety or movement. Essential tremor
What is the common treatment that individual may choose to treat Essential tremor? Alcohol, since it decreases tremor amplitude
Why is common to find alcoholism in a patient with Essential tremor? Alcohol- it decreases amplitude of the tremor
What is the medically relevant (not alcohol) treatment for Essential tremor? Non-selective B-blockers, and primidone.
Sudden, wild flailing of 1 arm, +/_ ipsilateral leg. Hemiballismus
What is Hemiballismus? Sudden, wild flailing of one arm, and possible the ipsilateral leg
Where is the lesion of Hemiballismus? Contralateral subthalamic nucleus
Contralateral or Ipsilateral lesion is seen in Hemiballismus? Contralateral
What type of movement disorder is seen with Cerebellar dysfunction? Intention tremor
What is Intention tremor? Slow, zigzag motion when pointing/extend toward a target
Slow, zigzag motion when pointing/extending toward a target Intention tremor
A person that extends on purpose the arm pointing to a target is seen with slow, zigzag movements of such extremity. Dx? Intention tremor
What is the definition of Myoclonus? Sudden, brief, uncontrolled muscle contraction
If the muscle contraction is sudden and brief, it is _______________. Myoclonus
What is the difference between the muscle contraction, in odr to be either dystonia or myoclonus? Brief contraction is myoclonus Sustained contraction is dystonia
What are two common examples of Myoclonus? Jerks and hiccups
Which organ failures are common to develop myoclonus? Liver and kidneys
How is Resting tremor defined? Uncontrolled movement of distal appendages; tremor alleviated by intentional movement.
What area is affected in Parkinson disease that leads to development of Resting tremor? Substantia nigra
Which type of tremor occurs at rest? Resting tremor
"Pill-rolling tremor" of Parkinson disease Resting tremor
How is Resting tremor alleviated? Producing intentional tremor
When is Restless legs syndrome the worst? During rest or nighttime
What are associated conditions that are seen with Restless legs syndrome? Iron deficiency and CKD
What is the treatment for Restless legs syndrome? Dopamine agonists
Which movement disease or condition is treated with Dopamine agonists, such as ropinirole and Pramipexole? Restless legs syndrome
What are two common Dopamine agonists used in Restless legs syndrome? Pramipexole and Ropinirole
Pathophysiology of FEVER: Cytokine activation during inflammation, such in an infection
What is the pathophysiology of a Heat stroke? Inability of body to dissipate heat
Incapacity to get rid of body heat. Heat stroke
What is the temperature for fever? < 40 C
> 40 C. Fever or Heat stroke? Heat stroke
What are the possible complications of fever? Febrile seizure
List of complication from Heat Stroke: - CNS dysfunction - End-organ damage - Acute respiratory distress syndrome - Rhabdomyolysis
What are the common drugs used to treat Fever? 1. Acetaminophen or ibuprofen for comfort 2. Antibiotic therapy if indicated
How is Heat Stroke managed? Rapid external cooling, rehydration and electrolyte correction
Decrease in cognitive ability, memory, or function with intact consciousness. Neurodegenerative disorder
What is the term given to dementia caused by depression? Pseudodementia
What mood disorder must be rule-out as the cause of dementia? Depression
What are some reversible causes of dementia? Hypothyroidism, vitamin B12 deficiency, neurosyphilis, normal pressure hydrocephalus, and depression.
What is the histologic and gross findings in Parkinson disease? 1. Loss of dopaminergic neurons of substantia nigra pars compacta 2. Lewy bodies: composed of a-synuclein
What are Lewy bodies composed of? a-synuclein
What are Lewy bodies? Intracellular eosinophilic inclusions found in Parkinson disease
What is MPTP? Contaminant in illegal drugs, is metabolized to MPP+, which is toxic to substantia nigra
What is a common toxic metabolic to the substantia nigra? MPP+, a metabolite of MPTP
TRAPSS Tremor Rigidity Akinesia Postural instability Shuffling gait Small handwriting
Medical term for small handwriting Micrographia
Autosomal dominant trinucleotide (CAG)n. Dx? Huntington disease
What is the gene involved in Huntington disease? Trinucleotide expansion of CAGn in the huntingtin (HTT) gene on chromosome 4
Which chromosome is affected in Huntington's disease? Chromosome 4
What is the common age range of onset of Huntington's symptoms? 20-50 years old
What are the symptoms seen with Huntington disease? Chorea, athetosis, aggression, depression, dementia.
Caudate loses ACh and GABA Huntington disease
What genetic phenomenon is associated with Huntington disease? Anticipation
Pathogenesis of Huntington's disease: Atrophy of caudate and putamen with ex vacuo ventriculomegaly
Neurotransmitter levels in Huntington disease: Increase dopamine, and decrease ACh and GABA
How do neurons "die" in Huntington disease? Via NMDA-R binding and glutamate excitotoxicity
GAGn repeat. Dx? Huntington disease
Which NTs are decreased in Huntington disease? ACh and GABA
Which neurotransmitter is elevated in Huntington disease? Dopamine
What is the MCC of dementia in the elderly? Alzheimer disease
Which trisomy condition is associated with a higher risk of developing Alzheimer disease? Down syndrome
Why are Down syndrome patients at higher risk of eventually developing Alzheimer disease? APP is located in chromosome 21
What neurotransmitter is decreased in Alzheimer's disease? ACh
What are some associated altered proteins of Alzheimer disease? 1. ApoE-2 2. ApoE-4 3. APP, presenilin-1, presenilin-2
What protein is associated with a decrease risk of sporadic form of Alzheimer disease? ApoE-2
A person with an altered ApoE-4 protein ----> Increase risk of sporadic form of Alzheimer disease
What proteins are associated with familial forms with earlier onset of Alzheimer disease? APP, presenilin-1, and presenilin-2
Widespread cortical atrophy, especially hippocampus. Narrowing gyri and widening of sulci. Gross findings of Alzheimer disease
What are some histological findings in Alzheimer disease? Senile plaques in gray matter: extracellular B-amyloid core
What are the Neurofibrillary tangles found in histology of Alzheimer disease patient? Intracellular, hyperphosphorylated tau protein = insoluble cytoskeletal elements
What part of the brain is most affected by Alzheimer disease? Hippocampus
What neurodegenerative condition is associated with Hirano bodies? Alzheimer disease
What are the intracellular rods found in Alzheimer disease? Hirano bodies
What are Hirano bodies? Intracellular eosinophilic proteinaceous rods in hippocampus
Where in the brain are Hirano bodies found? Hippocampus
What is the old name for Frontotemporal dementia? Pick disease
What is the new name of Pick disease? Frontotemporal dementia
What are the histological findings of Frontotemporal dementia? Inclusions of hyperphosphorylated tau or ubiquitinated TDP-43
Histological findings: Hyperphosphorylated Tau proteins and Ubiquitinated TDP-43. Dx? Frontotemporal dementia
What are clinical feurs to Lewy body dementia? Visual hallucinations, dementia with fluctuating cognition/alertness, REM sleep behavior disorder, and parkinsonism.
Where are Lewy bodies are primarily found? In cortex
What neurologic disorder is associated with REM sleep behavior disorder? Lewy body dementia
When is it called Lewy body dementia? If cognitive and motor symptom onset is < 1 year apart
If the cognitive deficit in is over a year apart form motor deficit, it is not longer considered Lewy body dementia, instead it is considered: Secondary to Parkinson disease
What is the cause of Vascular dementia? Result of multiple arterial infarcts and/or chronic ischemia
What is the second MCC of dementia in elderly? Vascular dementia
Step-wise decline in cognitive ability with late-onset memory impairment. Vascular dementia
MRI or CT show multiple cortical and/or subcortical infarcts. Dx? Vascular dementia
What do the images of a MRI or CT of vascular dementia patient most likely show? Multiple cortical and/or subcortical infarcts
What is Creutzfeldt-Jakob disease? Rapidly progressive dementia with myoclonus and ataxia
What is commonly seen in EEG of CJD? Periodical sharp waves
Important CSF finding in CJD? Incread 14-3-3 protein
Elevated 14-3-3 protein in CSF. Dx? Creutzfeldt-Jakob disease
- Rapid progressive dementia, + - "Startle myoclonus", + - Ataxia. Dx? Creutzfeldt-Jakob disease
Spongiform cortex. Dx? Creutzfeldt-Jakob disease
Prion neurodegenerative condition. Creutzfeldt-Jakob disease
What is another name for Idiopathic intracranial hypertension? Pseudotumor cerebri
Elevated ICP with no apparent cause on imaging. Dx? Idiopathic intracranial hypertension
What are conditions the cause increased ICP with no imaging evidence? Hydrocephalus, and obstruction of CSF outflow
What are common risk factor for Idiopathic intracranial hypertension? Female gender, Tetracycline, Obesity, vitamin A excess, and Danazol
What the common physical findings seen with Idiopathic intracranial hypertension? Headache , tinnitus, diplopia, no change in mental status
What is the treatment of Pseudotumor cerebri? Weight loss, acetazolamide, invasive procedures for refractory cases.
What cause the papilledema seen in Pseudotumor cerebri? Impaired optic nerve axoplasmic flow
What is accomplished by performing a LP on a patient with Idiopathic intracranial hypertension? Reveals elevated opening pressure and provides temporary headache relief.
What is the pathogenesis of Hydrocephalus? Increased CSF volume leads to ventricular dilation and possible increase in ICP
How are types of hydrocephalus mainly divided into? Communicating, Non-communicating,and Hydrocephalus mimics
WHat are the two types Communicating hydrocephalus? 1. Communicating hydrocephalus 2. Normal pressure hydrocephalus
What is the cause of Communicating hydrocephalus? Decreased CSF absorption by arachnoid granulations leading to development of elevated ICP, papilledema, and herniation
Arachnoid scarring post-meningitis is a common cause for: Communicating hydrocephalus
What population is most affected by normal pressure hydrocephalus (NPH)? Elderly
CSF pressure elevated only episodically; does not result in increased subarachnoid space volume; expansion of the ventricles. Dx? Normal Pressure Hydrocephalus
What is the result of the expansion of the ventiluces in NPH? Distorts the fibers of the corona radiata leading to development of trial do urinary incontinence, gait apraxia, and cognitive dysfunction.
What is the common triad associated with NPH? 1. Urinary incontinence 2. Gait apraxia 3. Cognitive dysfunction
What is another way to describe the gait apraxia seen in NPH patient? Magnetic gait
What part is distorted in NPH ventricle expansion leading to developing of its famous clinical triad? Fibers of the corona radiata
What is the treatment that often lead to reversivion of cysmtpos in NPH? CSF shunt placement
What type of hydrocephalus is classified as Obstructive? Non-communicating hydrocephalus
Caused by structural blockage of CSF circulation within ventricular system Pathogenesis of Non-communicating hydrocephalus
What are examples that cause the obstruction in Non-communicating hydrocephalus? - Stenosis of aqueduct of Sylvius - Colloid cyst blocking foramen of MOnro - Tumor
Ex vacuo ventriculomegaly Appearance of increased CSF on imaging, but is actually due to decreased brain tissue and neuronal atrophy
Apparent increase in CSF + Normal ICP + (-) NPH triad. Dx? Ex vacuo ventriculomegaly
What condition mimics a Hydrocephalus? Ex vacuo ventriculomegaly
Which conditions are often associated with Ex vacuo ventriculomegaly? Alzheimer's disease, advanced HIV, Pick disease, and Huntington's disease
Why are Huntington and Alzheimer's, and other neurodegenerative conditions associated with Ex vacuo ventriculomegaly? Decreased brain tissue and nurutonal atrophy
What is Multiple sclerosis? Autoimmune inflammation and demyelination of CNS with subsequent axonal damage.
Autoimmune inflammation and demyelination of CNS with subsequent axonal damage. Multiple sclerosis
What are the general features presented in Multiple sclerosis? 1. Acute optic neuritis 2. Brain stem/cerebellar syndromes 3. Pyramidal tract weakness 4. Spinal cord syndromes
Description Acute Optic neuritis in MS: Painful unilateral visual loss associated with Marcus Gunn pupil
What are the clinical symptoms of the brainstem/cerebellar syndromes seen with Multiple sclerosis? Diplopia, ataxia, scanning speech, intention tremor, nystagmus/INO.
INO deficit is often associated with _________ ___________. Multiple sclerosis
What is Lhermitte phenomenon? Electric shock-like sensation along spine on neck flexion
What neurological autoimmune condition is associated with Lhermitte phenomenon? Multiple sclerosis
What physical condition may exacerbate symptoms of Multiple sclerosis? Increased body temperature
What is the common pattern or characteristic of MS clinical course? Relapsing and remitting
Which population are most affected with Multiple sclerosis? Women in their 20s and 30s; more common in Caucasians living away from the equator
Oligoclonal bands are diagnostic for what condition? Multiple sclerosis
What is found in CSF sample of a Multiple sclerosis patient? Elevated IgG level aodn myelin basic protein
What is the gold standard test/imagining procedure for Multiple sclerosis? MRI
What are the findings in MRI of a MS patient? Periventricular plaques
MRI (+) periventricular plaques Multiple sclerosis
What are the periventricular plaques found in MRI of MS? Areas of Oligodendrocyte loss and reactive gliosis
Multiple white matter lesions disseminated in space and time. Dx? Multiple sclerosis
Multiple sclerosis affects white or gray matter? White matter
What is used to treat acute flares of MS? IV steroids
What is the treatment (chronic) for Multiple sclerosis? Stop relapses and halt/slow progression with disease-modifying therapies
What are common drugs denominated as Disease-modifying therapies? B-interferon, glatiramer, and Natalizumab
What is another name for Osmotic demyelination syndrome? Central pontine myelinolysis
What is Osmotic demyelination syndrome? Massive axonal demyelination in pontine white matter
What is the cause for Osmotic demyelination syndrome? Secondary to rapid osmotic changes, most commonly iatrogenic correction of hyponatremia but also rapid shifts of other osmolytes
Clinical features of Osmotic demyelination syndrome? Acute paralysis, dysarthria, dysphagia, diplopia, loss of consciousness
What is a possible severe complication of Osmotic demyelination syndrome? "Locked-in syndrome"
Problems arise from correcting serum Na+ too fast or too slow? Too fast
Excessively rapid correction of low serum Na+ to high serum Na+ leads to development of: Osmotic demyelination syndrome
Serum Na+ form high to low rapid correction lead to: Cerebral edema/herniation
Massive axonal demyelination in pontine white matter secondary to rapid osmotics changes, especially rapid correction of low serum Na+ to high serum Na+? Osmotic demyelination syndrome
What is the most common subtype of Guillain-Barre syndrome? Acute inflammatory demyelinating polyradiculopathy
What is acute inflammatory demyelinating polyradiculopathy? Autoimmune conditions associated with infections and motor fibers likely due to molecular mimicry, inoculation, and stress, but no definitive link to pathogens
What infection is associated to Acute inflammatory demyelinating polyradiculopathy? Campylobacter jejuni, virus (Zika) that destroy Schwann cells by inflammation and demyelination of peripheral nerves III-XII
What is the clinical presentation of Acute inflammatory polyradiculopathy, common subtype of GBS? 1. Symmetric ascending muscle weakness/paralysis and depressed /absent DTRs beginning in lower extremities 2. Facial paralysis (MC bilaterally) 3. Respiratory failure 4. +/- Autonomic dysregulation and/or sensory abnormalities
What is albuminocytologic dissociation? Increased CSF protein with normal cell count
What conditions is seen with albuminocytologic dissociation? Acute inflammatory demyelinating polyradiculopathy
What is the treatment for Acute inflammatory demyelinating polyradiculopathy? Disease-modifying treatment with plasmapheresis, and IV immunoglobulins
What is Acute disseminated (postinfectious) encephalomyelitis? Multifocal inflammation and demyelination after infection or vaccination
What is the common clinical presentation of Acute disseminated encephalomyelitis? Rapidly progressive multifocal neurological symptoms and altered mental status.
What is the common name for Hereditary motor an sensory neuropathy? Charcot-Marie-Tooth disease
What is Charcot-Marie-Tooth disease? Group of progressive hereditary nerve disorders related to the detective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath.
Defective production of proteins involved in the structures and function of peripheral nerves or myelin sheath. Dx? Charcot-Marie-Tooth disease
The defective protein in Charcot-Marie-Tooth disease lead to abnormal structure and function of which neurologic structure? Peripheral nerves and myelin sheath
What is a clinical relevant feature of Charcot-Marie-Tooth disease? Foot deformities
What are the common foot deformities in CMT disease? Pes cavus, and hammer toe
Mode of inheritance of Charcot-Marie-Tooth disease? Autosomal dominant
What are the symptoms seen in Charcot-Marie-Tooth disease? 1. Foot deformities 2. Lower extremity weakness, and 3. Sensory deficits
What is and what causes the most common type of Charcot-Marie-Tooth disease? CMT1A, is caused by PMP22 gene duplication
Duplication of gene PMP22 leading to synthesis of CMT1A. Dx? Charcot-Marie-Tooth disease
Demyelination of CNS due to destruction of oligodendrocytes secondary to JC virus infection. Dx? Progressive multifocal leukoencephalopathy
What type of patients is PML encephalopathy often seen? AIDS
What is the clinical course often seen by JC virus induced PML? Rapidly progressive and most often fatal
What areas of the brain are most commonly affected by PML? Parietal and occipital areas
What therapies or medication increase firsl of developing PML by JC viral infection? Natalizumab and Rituximab
What is a possible severe complication in AIDS patient by the reactivation of latent JC virus infection? Progressive multifocal leukoencephalopathy
Krabbe disease, Metachromatic leukodystrophy, and adrenoleukodystrophy, are examples of: Demyelinating disorders
List of Neurocutaneous disorders: 1. Sturge-Weber syndrome 2. Tuberous sclerosis 3. Neurofibromatosis type 1 4. Neurofibromatosis type 2 5. von Hippel-Lindau disease
What is another name for Sturge-Weber syndrome? Encephalotrigeminal angiomatosis
What is the cause of Sturge-Weber syndrome? Congenital, non-inherited, developmental anomaly of neural crest derivatives due to somatic mosaicism for an activating mutation in one copy of the GNAQ gene
What causes the "stain in face" in a patient with Sturge-Weber syndrome? Small (capillary-seized) blood vessels
What is the port-wine stain of the face in a Sturge-Weber syndrome? Nevus flammeus, a non-neoplastic "birthmark" in CN V1/V2 distribution
What are the clinical features of Sturge-Weber syndrome? 1. Port-wine stain of the face 2. Ipsilateral leptomeningeal angioma --> seizures/epilepsy 3. Episcleral hemangioma leading to increased IOP --> early-onset glaucoma
What is the affected gene in Sturge-Weber syndrome? GNAQ gene
What is the result of the elevated IOP of Sturge-Weber syndrome? Glaucoma
TSC1 mutation on chromosome 9 or TSC2 mutation on chromosome 16. Dx? Tuberous sclerosis
What are features of Tuberous sclerosis? - Hamartomas in CNS and skin - Angiofibromas - Mitral regurgitation - Ash-leaf spots - Cardiac Rhabdomyoma - Autosomal dominant inheritance - Mental retardation - Renal Angiomyolipoma - Seizures - Shagreen patches
What malignancies or tumors have increased incidence in Tuberous sclerosis, but not are part of the disorder? Subependymal giant cell astrocytomas and Ungual fibromas
Hamartomas in CNS and skin. Dx? Tuberous sclerosis
What kind of murmur is seen in Tuberous sclerosis? Mitral regurgitation
What AD neurocutaneous disorder is associated with cardiac rhabdomyoma and Ash-leaf spots? Tuberous sclerosis
What renal tumors are seen in Tuberous sclerosis? Renal angiomyolipoma
Shagreen patches are seen in : Tuberous sclerosis
What type of skin lesions or "stains" are seen in Tuberous sclerosis? Ash-leaf spots and Shagreen patches
How else is Neurofibromatosis type I? Recklinghausen disease
What chromosome of is affected in NF I? Chromosome 17
What are the most significant features of NF I? 1. Cafe-au-lait spots 2. Cutaneous neurofibromas 3. Optic gliomas 4. Pheochromocytomas 5. Lisch nodules
What condition is associated with Lisch nodules? Neurofibromatosis type I
What is Lisch nodules? Pigmented iris hamartomas
What are main clinical features of Neurofibromatosis type II? 1. Bilateral acoustic schwannomas 2. Juvenile cataracts 3. Meningiomas 4. Ependymomas
Which neurocutaneous conditions is associated with Bilateral acoustic schwannomas? Neurofibromatosis type II
What are the associated brain tumors of NF II? 1. Acoustic Schwannomas 2. Meningiomas 3. Ependymomas
Deletion of VHL gene of chromosome 3p. Dx? von Hippel-Lindau disease
What is the role of pVHL? Ubiquinates hypoxia-inducible factors 1
What are the characteristics of vHL disease? Numerous tumors, both benign and malignant
What mnemonic is sued by von Hippel-Lindau disease? HARP: - Hemangioblastomas with hyperchromatic nuclei in retina, brain stem, cerebellum, and spine - Angiomatosis - Bilateral Renal Carcinomas - Pheochromocytomas
Which organs and tissues develop hemangioblastomas in vHL disease? Retina, brain stem, cerebellum, and spine
What is the associated angiomatosis developing in vHL disease? Cavernous hemangiomas in skin, mucosa, and other organs
Bilateral renal carcinomas + Pheochromocytomas + Hemangioblastomas. Dx? von Hippel-Lindau disease
Created by: rakomi
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