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Neurology
FA complete review part 3.2 Pathology
| Question | Answer |
|---|---|
| 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 |
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rakomi
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