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194 Cards in this Set
- Front
- Back
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Essential tremor
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Bilateral arm involvement
Body affected by tremor: arms mostly then head, legs, larynx and trunk. Tremor associated with purposeful movement Immediate latency period. |
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Cogwheel rigidity
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Feeling of periodic resistance to passive movement felt by the examiner in a limb.
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Bradykinesia
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Slowed ability to start and continue movements and impaired ability to adjust the body’s position
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Lead-pipe rigidity
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The hypertonicity felt in a parkinsonian limb throughout the range of movements of a joint. It is indicative of increased tone in all the sets of muscles around a joint.
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Physiological tremor
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A very-low-amplitude fine tremor (between 6 Hz and 12 Hz) that is barely visible to the naked eye. It is present in every normal individual during maintaining a posture of movement. Neurologic examination results of patients with physiologic tremor are usually normal.
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Enhanced physiological tremor
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High-frequency, low amplitude, visible tremor that occurs primarily when a specific posture is maintained. Drugs and toxins induce this form of tremor
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Chorea
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Sudden jerky irregular movements with muscle contraction that are not repetitive or rhythmic but appear to flow from one muscle to the next.
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Athetosis
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Twisting and writing movements often associated with chorea
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Dystonia
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Sustained muscle contractions cause twisting and repetitive movements or abnormal postures.
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Tardive dyskinesia
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Neurologic d/o caused by the long-term and/or high dose use of dopamine antagonists, usually antipsychotics and among them especially the typical antipsychotics. It is an impairment of voluntary movement that continues or appear even after the drugs are no longer taken.
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Myoclonus
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Sudden, involuntary jerking of a muscle or group of muscles.
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Opisthotonos
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Great rigid spasm of the body with the back fully arched and the heels and head bent back.
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Substantia nigra
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Heterogenous portion of the midbrain and a major element of the basal ganglia system. It consists of the pars compacta, pars reticulata and pars lateralis.
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Lewy body
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As eosinophilic, round inclusion found in the cell cytoplasm of substantia nigra, the nucleus basalis of meynert, locus ceruleus, dorsal raphe, and the dorsal motor nucleus of cranial nerve X. they contain alpha-synuclein, a presynaptic protein, the function of which is unknown. Neurofilament proteins and ubiquitin are other important constituents of lewy body.
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Parkinson Disease
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Unilateral tremor, associated with stooped posture, shuffling gait, memory loss.
Tremor at rest Latency – several seconds (longer than essential tremor) |
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Multiple system atrophy
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2 major forms MSA-P resembles Parkinson except that the tremor is less prominent, and the disorder tends to be quite symmetric.
MSA-C also called olivopontocerebellar atrophy, presents as a cerebellar syndrome. Both forms have prominent autonomic insufficiency – including orthostatic hypotension and impotence. Conventional MRI usually show abnormalities. |
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Dementia with lewy bodies
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Early onset dementia
Delusions and hallucinations Fluctuations in consciousness and myoclonus |
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Corticobasalganglionic degeneration
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Typically has unilateral, coarse tremor, rigidity, increased reflexes as well as limb apraxia/limb dystonia/alien limb phenomenon.
Disorder is the only one that typically has the asymmetric appearance of parkinson disease. |
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Progressive supranuclear palsy
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Characterized by supranuclear downgaze palsy (inability to voluntarily look down) and square wave jerks on extraocular motion testing. Patients typically have an upright rather than flexed posture. Frequent falls can be an early finding.
Tremor is not common and there is pseudobulbar emotionality. |
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Levodopa
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Crosses the BBB.
Converted to dopamine in the brain, peripheral breakdown in the gut is inhibited by the addition of inhibitors of aromatic amino acid decarboxylase, carbidopa. Can be broken down peripherally by catechol-O-methyltransferase (COMT). |
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Dopamine agonists
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Cross the blood-brain barrier and act directly as primarily D2-type receptors without requiring conversion. These agents include: pramipexole, ropinirole and bromocriptine.
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MAO-B inhibitors
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Selegiline and rasagiline can improve symptoms in both patients with mild disease (as monotherapy) and patients already on levodopa therapy. Anticholingerics such as trihexyphenidyl or diphenhydramine (Benadryl) are used primarily to combat tremor, but have many side effects especially in older individuals.
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Amantadine
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Acts primarily by blocking glutamate N-methyl-D-aspartate (NMDA) receptors and has a mild attenuation of the cardinal symptoms of resting tremor and dystonia. Recently, amantadine has been show to help alleviate levodopa induced dyskinesias.
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Ataxia
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An unsteady and clumsy motion of the limbs or torso caused by a failure of the gross coordination of muscle movements.
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Trinucleotide repeat expansion disease
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Caused by stretches of DNA in a gene that contain the same trinucleotide sequence repeated many times. These repeats are a subset of unstable microsatellite repeats that occur throughout all genomic sequences.
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Dyskinetic disorders
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Acute: acute dystonia, acute akathisia, drug-induced parkinsonism
Chronic: Common: tardive dyskinesia, tardive dystonia, tardive akathisia, perioral tremor (rabbit syndrome) Uncommon: tardive myoclonus, tardive tics, tardive tremor Miscellaneous: neuroleptic malignant syndrome |
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Epidural hematoma
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A collection of blood between the inner table of the skull and dura. It is considered to be an extra-axial hematoma and appears to have a biconvex shape on CT scan of the head
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Subdural hematoma
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Also considered to be an extra-axial hematoma. It is a collection of blood between the brain and the dura. Appears as a concave shape on CT scan of the head.
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Delirium
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Neurobehavioral disorder with a fluctuating course entailing inattention and acute alteration in mental status.
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Glasgow Coma Scale
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Delineates categories of head injury and levels of consciousness in patients with traumatic brain injury. Scale divided into 3 categories: eye opening, verbal response and motor response. Maximum score is 15 and minimum score is 3.
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Subarachnoid hemorrhage
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Hemorrhage within the subarachnoid space tha tis caused by rupture of an aneurysm, arteriovenous malformation, neoplasm, angioma, cortical thrombosis, mycotic aneurysm tear, spread of blood or dissection from an intraparenchymal hemorrhage or trauma.
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Babinski sign
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Extension of the big toe followed by abduction of the other toes when the lateral sole of the foot is timulated. It is performed by stroking the foot at the heel and moving the stimulus toward the toes. It is a sensitive and reliable sign of cortical spinal tract disease. Aka plantar reflex.
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Attention
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Ability to focus on specific stimuli while excluding others.
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Delirium risk factors
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Elderly (>80 yo)
Male > female Preexisting cognitive impairment; number and severity of medical illnesses Dehydration/electrolyte disturbances; infections: urinary/pulmonary Hypoxemia/cardiorespiratory failure; malnutrition Drug abuse: EtOH or hypnotic dependency; sleep disturbance Fever/hypothermia; polypharmacy/analgesic use Depression; fractures Physical trauma; burns Sleep disturbance; visual/auditory impairment. |
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Ischemic stroke
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Cerebral infarction associated with neurologic symptoms of greater than 24-hour duration
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Transient ischemic attack
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A cerebral ischemic even associated with focal neurologic deficits lasting less than 24 hours and generally no evidence of cerebral infarction.
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Intracerebral hemorrhage
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A cerebrovascular even characterized by arterial rupture and parenchymal hemorrhage
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Homonymous hemianopia
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Loss of one-half of the field of view on the same side in both eyes
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Todd’s paralysis
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Brief period of transient (temporary) paralysis following a seizures.
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Subarachnoid space
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Spongy interval between the arachnoid mater and the pia mater. The headache and nuchal rigidity is caused by chemical inflammation of the pia arachnoid from blood degradation products in this space.
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Sentinel bleed
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Intermittent aneurysmal subarachnoid hemorrhage causing lesser headaches that precede the ‘worst headache’ that occurs with rupture of the aneurysm
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Vasospasm
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Most alarming complication of aneurysmal subarachnoid hemorrhage in which irritation cause constriction of major cerebral arteries, vasospasm lethargy, and cerebral infarction. Vasospasm occurs mostly with aneurysms rather than other causes of subarachnoid hemorrhage and peaks after 4 to 14 days. Transcranial doppler can be used to detect a change in flow velocity in an affected middle cerebral artery.
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Acute communicating hydrocephalus
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Complication that occurs because of obstruction of the subarachnoid granulations in the venous sinuses by the subarachnoid blood. CT shows enlarged lateral, third and fourth ventricles, with clinical signs of headache, vomiting, blurry and double vision, somnolence and syncope.
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Endovascular coiling
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Indicated to reduce rebleeding in low grade cases of subarachnoid hemorrhage and has been shown to be superior to clipping
Treatment of subarachnoid hemorrhage |
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Clipping
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Treatment of subarachnoid hemorrhage
Should be performed in the first 48 hours after onset or be delayed for 2 weeks to avoid the window of greatest risk for vasospasm, especially with complicated high-grade cases. |
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Triple H therapy
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Treatment of subarachnoid hemorrhage
Mainstay of medical management to reduce vasospasm. Hypertensive Hypervolemic Hemodilution To maintain cerebral perfusion and nimodipine a CCV. |
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Carotid dissection
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Tear in the carotid arterial wall tha can result in luminal obstruction, thromboembolic complications, and/or pseudoaneurysm formation. Dissections can also occur in the vertebral arteries, or less commonly, the large intracranial arteries.
Often present with orbital or neck pain associated with neurologic findings. Horner syndrome or ipsilateral dissected carotid side can accompany these symptoms. Trauma to the neck or vigorous movements to the neck will often trigger the dissection. |
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Patent foramen ovale
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Persistent opening in the interatrial septum associated with paradoxical embolism in patients with cryptogenic stroke
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Arteriovenous malformation
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Congenital high-pressure, high-flow cerebral vascular malformation characterized by direct arteriovenous shunting.
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Seizure
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Temporary, self-limited cerebral dysfunction as a result of abnormal, self-limited hypersynchronous electrical discharge of cortical neurons. There are many kinds of seizures, each with characteristic behavioral changes and each usually with particular EEG recordings.
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Therapy for simple and complex partial seizures
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Carbamazepine
Valproate Gabapentin Lamotrigine Topiramate phenytoin |
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Therapy for secondary generalized seizures
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Carbamazepine
Valproate Gabapentin Lamotrigine Topiramate phenytoin |
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Therapy for tonic-clonic seizures
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Valproate
Lamotrigine Topiramate Carbamazepine phenytoin |
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Therapy for absence seizures
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Valproate
Lamotrigine Ethosuximide Zonisamide |
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Therapy for myoclonic seizures
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Valproate
Clonazepam levetiracetam |
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Therapy for tonic seizures
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Valproate
Felbamate Clonazepam zonisamide |
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Absence seizures
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Absence seizures are brief episodes of starting that usually occur in childhood and last 5 to 10 second. If the seizure lasts beyond 10 seconds, ther can also be eye blinking and lip smacking. Usually occur in clusters and occur dozens or hundred of times a day
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Complex partial seizure
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Previously called temporal lobe seizures or psychomotor seizures. Complex refers to loss of consciousness and lack of awareness of the patient’s surroundings. Patients often engage in repetitive behavior, known as automatisms. This is the most common type of seizure in adults with epilepsy. Patients appear to be awake but do not respond normally to their environment. The ‘spell’ usually lasts less than 3 minutes and can be immediately preceded by a simple partial seizure.
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First aid for seizures
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Do not restrain the person
Remove dangerous objections from the person’s path. Calmly direct person to sit down and guide him or her from dangerous situations. Use force only in an emergency to protect the person from immediate harm. Observe, but do not approach a person who appears angry or combative Remain with the person until he or she is fully alert. |
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Syncope
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Sudden brief loss of consciousness
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Orthostatic syncope
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Syncope associated with a sudden change in position from supine to sitting up or sitting to standing up
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Electroencephalography
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The neurophysiologic measurement of the electrical activity of the brain by recording from electrodes placed on the scalp or, in special cases, subdurally or in the cerebral cortex.
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Epilepsy
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Neurologic condition that makes people susceptible to seizures. A seizure is a change in sensation, awareness, or behavior brought about a brief electrical disturbance in the brain.
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Tilt-table testing
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Test to evaluate how the body regulates blood pressure in response to some very simple stresses while lying on a special table. It involves cardiac monitoring (ECG), blood pressure monitoring, and IV infusion of drugs to stress the system.
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Sick sinus syndrome
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Type of bradycardia in which the SA or sinus node is not working as it should.
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Vasovagal syncope
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Is not a serious or life-threatening condition but is an abnormal reflex. Results in drop in blood pressure leading to a decrease of blood flow to the brain resulting in dizziness or fainting.
Pt usually upright and describes a sensation of lightheadedness, dimmed vision and hearing, depersonalization, sweating, nausea and increased heart rate. |
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Pseudoseizures
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An attack resembling an epileptic seizure but having purely psychological causes; it lacks the electroencephalographic characteristics of epilepsy and the patient may be able to stop it by an act of will.
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Postcoital cephalgia
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Occurs both before and after orgasm. It is seen equally in men and women. Head pain is usually sudden, often pulsatile, and can involve the entire head. Fewer than 2% of patients who are seen with subarachnoid hemorrhage have the hemorrhage occur during intercourse.
Pts should be pretreated prior to sexual relations with medication, usually a simple analgesic. |
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Pseudotumor cerebri
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(benign intracranial HTN)
is manifested by increased intercranial pressure without evidence of a CNS malignancy. Pts with benign intracranial HTN complain primarily of Has often associated with visual disturbances. Usually seen in female pts who are obese and often have menstrual irregularities. |
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Acute glaucoma
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Often characterized by sudden orbital or eye pain in the face of nausea and vomiting. Pain can begin after the use of anticholinergic drugs.
Hallmark – elevated intraocular pressure |
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Brain tumor
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Often associated with headaches.
Often present as typical tension or migraine HA. Can be quite frequent and can occur on a daily basis, often awakening a patient from sleep. Neuro exam can be normal but can reveal focal abnormalities as wel as papilledema on funduscopic exam. HA – presenting feature in ~ 40% of brain tumors. |
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Abortive treatment of migraine
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4 medications used for abortive therapy
1) Triptans 2) Ergotamine 3) Dihydroergotamine 4) Isometheptene mucate, dichloralphenazone, acetaminophen (midrin) |
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Triptans
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Used for abortive treatment of migraine.
Work at the 5HT-1D serotonin receptor agonists. 80% effective in the treatement of an individual migraine attack. Should be used early in HA often during the prodrome but also be used on HA has started. Drugs can be repeated as early as 4 hrs in HA reoccurrence but no more than 3 doses per 24 hrs should be used. S/E: nausea, vomiting, numbness and tingling of the finger sand toes. Contraindicated in CAD or HTN, or if the pt has hemiplegia or blindness as an aura. Sumatriptan (imitrex) Almotriptan (axert) Rizatriptan (maxalt) Zolmitriptan (zomig) Eletriptan (relpax) Naratriptan (amerge) Frovatriptan (frova) |
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Common migraine triggers
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Chocolate, cheese, red wine, citrus fruits, coffee, tea, tomatoes, potatoes, irregular meals
Excessive or insufficient sleep. Changes in hormone balance in women (such as menses, the pill or menopause) Stress or relaxation after a period of stress Caffeine withdrawal Physical activity Smoking Flashing lights or noise Weather- high pressure conditions, changes in pressure, hot dry winds, change of season, exposure to sun and glare Sexual arousal Smells – paint, fumes from car heaters or perfume. |
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Ergotamine derivatives
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Should be considered for migraine with patients fail triptans.
Usually a 2mg sublingual tablet is prescribed and repeated for 2 doses each separated by 30 minutes if necessary. Dose can be repeated 3 times a day. Should not be prescribed on an ongoing daily basis for chronic usage. |
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Dihydroergotamine
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An episodic migraine, can become more chronic and intractable, can respond to intramuscular or IV dihydroergotamine (DHE). Initial dose is 0.5 mg IV with 10mg of metoclopramide or 5 mg prochlorperazine if nausea is present.
If the HA improves, the dosage of DHE plus metoclopramide is repeated for 2 or more doses separated by 8 hours and then one more dose of DHE alone. Nasal spray formation now available. |
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Midrin
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Spans the gap between abortive and prophylactic therapy.
Consists of: acetaminophen, dichloralphenazone (muscle relaxant), and isometheptene mucate (vasoconstrictor). |
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Vascular headache
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Type of headache, including migraine, throught to involve abnormal function of the brain’s blood vessels or vascular system.
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Paraphasic errors
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Production of unintended syllables, words or phrases during the effort to speak
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Nucleus basalis of meynert
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Group of nerve cells that has wide projections to the neocortex and is rich in acetylcholine and choline acetyltransferase.
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Pseudodementia
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Severe form of depression resulting from a progressive brain disorder in which cognitive changes mimic those of dementia
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Multi-infarct dementia
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Dementia in the setting or cerebrovascular disease, occuring after multiple cerebral infarctions, whether large of small (lacunar).
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Constructional apraxia
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Difficulty in performing tasks involved with construction, for example, drawing a five-pointed star
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Executive function
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Mental capacity to control and plan mental skills, the ability to sustain or flexibly direct attention, the inhibition of inappropriate behavioral or emotional responses, the planning of strategies, and the ability to flexibly switch among problem-solving strategies. It is mediated by the prefrontal lobes of the cerebral cortex.
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Ideomotor apraxia
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Disturbance of voluntary movement in which a person cannot translate an idea into movement
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REM-related disorders
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Parasomnia involving dissociation of the characteristic stages of sleep. The major feature is loss of motor inhibition leading to a wide spectrum of behavioral release during sleep, that is ‘acting out dreams’
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Diffuse lewy body dementia
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Felt to represent the second most common cause of dementia in developed countries in the western hemisphere
10-20% of dementias Men are more affected than women Usual onset is in late 50’s and beyond |
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Optic neuropathy
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Unilateral or bilateral impairment of optic nerve function. The diagnosis is usually made clinically. Ddx: congenital, hereditary, infectious, inflammatory, infiltrative, ischemic, demyelinating (optic neuritis), compressive etiologies.
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Optic neuritis
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General term for idiopathic, inflammatory, infectious or demyelinating optic neuropathy
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Anterior optic neuritis
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Papillitis
Swelling of the optic nerve |
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Retrobulbar neuritis
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Also called posterior optic neuritis. Patients have symptoms because of compromise of the optic nerve, but the optic nerve appears normal.
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Acute disseminated encephalomyelitis
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ADEM is an acute, uniphasic syndrome, probably caused by immune-mediated inflammatory demyelination. It often is associated with immunization, vaccination, or postviral illness.
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Acute necrotizing hemorrhagic encephalomyelitis
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ANHE – is a hyperacute form of ADEM with similar symptoms and cause.
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Meningitis
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Inflammation of the membranes of the brain and spinal cord (meninges) caused by many different organisms
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Kernig sign
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Inability to completely extend the leg when the hip is flexed in the supine position. Caused by severe stiffness in the hamstrings muscles from inflammation of the lumbosacral roots.
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Brudzinski sign
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Flexion of the neck causes involuntary flexion of the thighs and the legs.
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Latex particle agglutination
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Test where an antibody or antigen coats the surface of latex particles (sensitized latex). When a sample containing the specific antigen or antibody is mixed with the milky appearing sensitized latex, visible agglutination is noted. Used to detect H. influenzae type b, s. pneumoniae, and n. meningitidis A, B and C soluble antigens.
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HSV PCR
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PCR is a molecular technique that allows a small amount of DNA to be replicated and amplified. In this case HSV DNA is detected. HSV PCR for CSF has an estimated sensitivity of 95% and specificity of almost 100%.
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EV RT-PCR
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Enteroviral reverse transcriptase PCR is a technique in which cDNA is then replicated and amplified through standard PCR protocols. In this particular case various viruses belonging to the enterovirus family can be detected.
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Nystagmus
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Rapid involuntary oscillatory movement of the eyes.
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Infantile botulism
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A neuroparalytic disease presenting in a subacute manner caused by C. botulinum toxin type A and B that typically occurs when ingested spores germinate and colonize in the GI tract of the infant.
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Dysautonomia
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Dysfunction of the autonomic nervous system manifested by tachycardia, bradycardia, hypotension, hypertension, hyperthermia, hypothermia, blurred vision, xerostomia, constipation, diarrhea, bladder urgency, bladder hesitancy, erectile dysfunction, hyperhidrosis, or anhydrosis.
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Sporadic CJD
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Disease appears even thought the person has no known risk factors for the diseases. This is by far the most common type of CJD and accounts for at least 85% of cases
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Hereditary CJD
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Person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. Approximately 5-10% of cases of CJD in the US are hereditary.
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Acquired CJD
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Disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient.
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Creutzfeldt-Jakob Disease
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Rare degenerative, invariable fatal brain disorder. It affects approximately one person in every one million per year worldwide. Appears in later life and runs a rapid course. Typically onset of symptoms occurs approximately at 60 yo and ~ 90% of patients die within 1 year. In early stages of disease patients can have failing memory, behavioral changes, lack of coordination, and visual distrubances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities and coma can occur.
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Tabes dorsalis
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Neurologic form of syphilis
10-20 yrs after initial infection Lancination pain, sensory ataxia, bowel dysfunction, bladder dysfunction or cranial nerve abnormalities. |
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Syphilitic meningitis
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1-2 yrs after initial infection
Cranial mononeuropathies, hydrocephalus and focal hemispheric signs. |
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Argyll robertson pupils
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Small pupils that constrict when focusing but fail to constrict when exposed to brigh light (accommodate but do not react.
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EMG/nerve conduction studies
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Evaluates the integrity of the peripheral nerve and evaluates various electrical muscle properties allowing the clinical to determine the presence of either a muscle or nerve disorder.
Test is useful in evaluating primarily the peripheral nervous system. |
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H reflex
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Electrical equivalent – to a mono-synaptic stretch reflex. It often reflects pathology along the afferent and efferent fibers and/or the dorsal root ganglion.
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Lancinating pain
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A sensation of piercing, stabbing or cutting
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Ptosis
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Droopiness of the eyelids
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Romberg sign
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Falling over when a person is standing with eyes closed, feet together, and hands in the outstretched position
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Toxoplasmosis
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Caused by T. gondii
Leading cause of focal CNS disease in AIDS and is most frequently seen during the later phases of the diseases. Acquired in pregnancy can cause various congenital anomalies in the fetus including hydrocephalus, intracerebral calcification, retardation, chorioretinitis, hearing loss and even death. Clinical features: HA and constitutional symptoms early on. Confusion, drowsiness, focal weakness, aphasia and seizures later on in the course. Coma can ensue within days to weeks if no treatement is started. Radiculomyelopathy can occasionally be present. Other features: ataxia, cranial nerve palsies, hemianopia, personality changes. |
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Generalized tonic-clonic seizure
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It is often referred to as a grand mal seizure and involves loss of consciousness, violent muscle contractions and rigidity
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Folinic acid
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Reduced form of folic acid that does not require reduction reaction by enzyme for activation
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Radiculomyelopathy
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Process affecting the nerve root and spinal cord
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Ring-enhancing lesion
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Lesion that shows peripheral enhancement with central hypodensity after being administered contrast.
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Papilledema
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Disk edema from raised intracranial pressure; commonly bilaterally
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Increased intracranial pressure
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CSF pressures above 200 mm of water in a nonobese pt or greater than 250 mm of water in obese patients
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Herniation syndromes
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Downward displacement of brain tissue when intracranial pressure in the supratentorial compartment reaches a certain level
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Hydrocephalus
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Abnormal excessive accumulation of cerebral spinal fluid in the brain
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Proptosis
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Abnormal protrusion of the eyeball
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Audiogram
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Test that measures the level of hearing in each ear
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Bell palsy
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An idiopathic form of facial paralysis, thought to be caused by herpes simplex reactivation
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Cholesteatoma
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Benign tumor composed of epithelial debris from the tympanic membrane that becomes trapped in the middle ear
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Facial nerve electroneurogram
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Electrical test that evokes a compound muscle action potential (cMAP) by stimulating the facial nerve. The ENoG uses surface electrodes rather than needles to measure the cMAP. Each side is stimulated at the stylomastoid foramen, and the responses from muscle groups are measured and compared. Significant nerve damage is indicated by a 90% or greater reduction in the cMAP.
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Otorrhea
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Drainage from the ear
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Postherpetic neuralgia
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Neuropathic pain resulting from resolved herpes infection
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Tzanck smear
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Test that looks for intracytoplasmic particles due to viral infection
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Vesicles
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Small fluid-filled blisters on an erythematous base.
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Etiologies of ptosis
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Local mechanical lid abnormalities- thyroid disease, ocular surgery, infiltrative processes (sarcoid, amyloid), orbital cellulitis, primary or metastatic tumors
Myopathy – mitochondrial cytopathies (kearns-sayre), congential (centronuclear myopathy), oculopharyngeal muscular dystrophy Neuromuscular junction – m. gravis, botulism Oculosympathetic – horner syndrome; associated miosis 3rd nerve palsy- ischemic, metabolic (DM), uncal herniation syndrome, posterior communicating artery aneurysm, cavernous sinus; associated with other cranial nerve abnormalities. 3rd nucleus- ischemic Supranuclear – midbrain neoplasms (bilateral), contralateral middle cerebral artery ischemia |
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Anti-MuSK antibodies
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Muscle-specific receptor tyrosine kinase antibodies. MuSK is a surface membrane enzyme critical for aggregating acetylcholine receptors during neuromuscular junction development. It is often seen in individuals who are seronegative for acetylcholine antibodies.
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Dysarthria
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Speech disorder arising from weakness, paralysis or incoordination of speech musculature.
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Myogenic
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Disorder of muscle or muscle tissue
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Neurogenic
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Disorder affecting either anterior horn cell, nerve root, plexus or peripheral nerve
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Mitochondrial cytopathies
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Diverse group of diseases affecting the mitochondria.
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Benign paroxysmal positional vertigo
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Recurrent vertigo and nystagmus occurring when the head is placed in certain positions, usually not associated with lesions of the CNS.
Duration of vertigo: 5- 30 seconds Repeated attacks over weeks, months or years PE: geotropic nystagmus with reversibility and fatigue on Dix-Hallpike testing Treatment: canalith repositions procedure, brandt-daroff exercises |
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Otolith
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Minute calciferous granules within the gelatinous membrane of the saccule and utricle. The otoliths, begin heavier than the surrounding fluid, render the otolith organs sensitive to changes in position with respect to gravity.
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dix-hallpike test
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Maneuver that provokes the nystagmus and vertigo associated with benign paroxysmal positional vertigo.
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Canalith repositioning procedure
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Therapeutic maneuver that moves misplaced otoliths from the posterior semicircular canal into the saccule
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Brandt-daroff exercises
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Therapeutic series of maneuvers that dissipate misplaced otoliths and that habituates the vertigo from these otoliths.
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Vestibular migraine
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Duration of vertigo attacks: seconds to minute
Duration of symptoms: minutes to hours, recurrent attacks Associated symptoms: HA, scintillations, other neurologic symptoms (weakness or numbness of extremity, changes in speech….) PE: usually normal Treatment: either suppressive or abortive therapy for migraine, refer to neurologist |
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Vestibular epilepsy
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Duration of vertigo attacks: seconds to minutes
Duration of symptoms: minutes, recurrent episodes Associated symptoms: loss of consciousness, other neurologic symptoms PE: usually normal Treatment: anti-epileptic therapy, refer to neurology |
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Vertebrobasilar insufficiency
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Duration of vertigo attacks: seconds to minutes
Duration of symptoms: repeated episodes or evolving symptoms over weeks Associated symptoms: changes in speech or swallowing, cerebellar symptoms, history consistent with atherosclerosis PE: can be normal, might have cranial nerve findings, cerebellar signs or carotid bruit Treatment: anti-platelet therapy, management of risk factors. |
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Meniere disease
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Duration of vertigo attacks: 20 minutes to 24 hours
Duration of symptoms: repeated episodes over weeks to months to years Associated symptoms: aural pressure, low pitch (roaring) tinnitus, low frequency hearing loss. PE: nystagmus during an attack, normal ear examination, might have hearing loss on audiogram, vestibular weakness on ENG Treatment: low salt diet and diuretic therapy, might need vestibular suppressants, might vestibular ablative or nerve section. |
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Viral vestibular neuronitis
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Duration of vertigo attacks: 24 hours +
Duration of symptoms: several days, resolving over a few weeks as vestibular compensation occurs Associated symptoms: nausea and vomiting PE: horizontal nystagmus when patient is seen early in course, otherwise normal examination, vestibular weakness on ENG. Treatment: supportive care, anti-emetics, possibly vestibular suppressants, might need vestibular rehabilitation therapy |
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Acute suppurative labyrinthitis
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Duration of vertigo attacks: 2-3 days
Duration of symptoms: several days, resolving over a few weeks as vestibular compensation occurs. Associated symptoms: severly ill with nausea and vomiting, hearing loss, tinnitus, otorrhea PE: otitis media or cholesteatoma, nystagmus early in presentation, possibly facial paralysis from cholesteatoma Treatment: IV antibiotics and possibly surgery. |
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Acute serous labyrinthitis
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Duration of vertigo attacks: 2 – 3days
Duration of symptoms: several days, resolving over a few weeks as vestibular compensation occurs Associated symptoms: hearing loss, tinnitus, nausea and vomiting PE: history of past otologic surgery, nystagmus early in presentation Treatment: supportive care with anti-emetics, possible vestibular suppressants, might need vestibular rehabilitation, use steroids if hearing loss is present |
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Herpes zoster oticus
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(ramsay hunt syndrome)
Duration of vertigo: 2-3 days Duration of symptoms: acute illness lasts approximately 7 -10 days, residual symptoms can be long lasting. Associated symptoms: herpetic rash in ear, retro-auricular pain, hearing loss PE: facial paralysis, hearing loss Treatment: antiviral medications, steroids, might need vestibular rehabilitation. |
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Chronic inflammatory demyelinating polyneuropathy
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An acquired peripheral neuropathy with an extremely variable clinical presentation and course.
At onset pts usually present with a generalized pattern of numbness and weakness in upper and lower extremities and spontaneous pain that develops gradually over several weeks. Progressive sensory ataxia, motor deficits. Proximal and distal limbs are commonly affected ina roughly symmetrical pattern. Can develop gradually or rapidly (being mistaken for guillain-barre). |
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Demyelinating
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Immune-mediated damage to myelin surrounding the peripheral nerves, spinal roots, and cranial nerves resulting in clinical weakness and numbness, and electromyographic evidence of profoundly delayed or absent nerve conduction velocities.
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Flaccid
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Lower motor neuron weakness with hypo- or areflexia, hypotonia and, in the case of chronic disease, muscle atrophy
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Dermatomyositis
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Differs from polymyositis by its immunopathogenesis but also by the involvement of skin, with rash, discoloration and tissue calcification.
Subacute onset of proximal muscle pain and weakness, some dysphagia. Proximal muscle weakness. |
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Heliotrope rash
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Bluish-purple discolorations on the face, lids, neck, shoulders, upper chest, elbows, knees, knuckles and back of patients with dermatomyositis
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Gottron nodules
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Flat-topped raised nonpruritic lesions found over the dorsum of the MCP, PIP and DIP joints
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Anti-Jo-1 antibody
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Antibody that recognizes a cytoplasmic histidyl transfer RNA synthetase
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Raynaud phenomenon
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Condition resulting rom poor circulation in the extremities (fingers and toes) in a person with hish or her skin is exposed to cold or the person becomes emotionally upset, the blood vessels under the skin spasm, and the blood flow slows. Called vasospasm. These areas can become cyanotic and cold.
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Calcinosis cutis
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Development of dystrophic calcification in the soft tissues and muscles, leading to skin ulceration, secondary infection and joint contracture. Occurs in up to 40% of children with dermatomyositis and less commonly in adults.
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Inclusion body myositis
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More gradual onset of weakness
Muscle weakness is proximal, distal muscle groups can also be affected, and asymmetry of involvement is characteristic. Atrophy of the deltoids and quadriceps is often present, and weakness of forearm muscles (especially finger flexors) and ankle dorsiflexors is typical. Peripheral neuropathy with loss of deep tendon reflexes can be present in some patients. |
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Idiopathic inflammatory myopathies
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Inclusion body myositis: greater than 50 years, males, poor therapeutic response. Biopsy findings: vacuoles, amyloid deposits
CK level is less than 10x normal. No rash or malignancy association, fmhx rare. Polymyositis – adult, females, slight association with malignancy. CK level 50x normal. Variable therapeutic response. Biopsy: inflammatory complement deposits. No rash or fam history. Dermatomyositis – all ages, females, associated with malignancy, rash, CK level is 50x normal. Biopsy shows inflammation. Good therapeutic response. No family history association. |
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Upper motor neuron disease
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Pathologic process resulting in skeletal muscle weakness, spasticity, and increased reflexes with normal sensation
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Lower motor neuron disease
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Pathologic process resulting in skeletal muscle weakness, flaccidity, decreased or absent reflexes, muscle atrophy and fasciculations with normal sensation.
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Myelopathy
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Pathologic process that is extrinsic or intrinsic to the spinal cord, which can result in muscle weakness, spasticity and sensory abnormalities at and below the level of cord pathology.
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Radiculopathy
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Pathologic process affecting the motor and/or sensory nerve roots, which originate from or enter to the spinal cord; usually caused by compression or narrowing of nerve root foramen (nerve/root canal) associated with degenerative spine or disc disease (spondylosis or spondylolisthesis)
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Carpal tunnel
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Narrow, rigid passageway or ligament and bones at the base of the hand through which the median nerve travels.
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Tinel sign
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Reproduction of numbness, tingling, or pain with tapping or pressing on the median nerve at the level of the patient’s wrist
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Phalen sign
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Test involves having the patient hold his or her forearms together, the presence of CTS is suggested if one or more symptoms such as tingling or increasing numbness, is felt in the fingers within 1 minute.
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Cervical radiculopathy
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Results from mechanical nerve root compression or intense inflammation of nerve root (s) resulting in an acute shooting pain and/or weakness in the distribution of that nerve root.
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Brachial plexopathy
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Bundle of nerves that lies between the neck and the axilla with the distal portion lying behind the clavicle and the pectoral muscles. It is formed form C5-T1 nerve roots and is best understood by dividing it into three parts; trunks, divisions and cords.
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Generalized epilepsy
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Recurrent seizures that arise from both cerebral hemispheres at once because of an inherited predisposition. These include absence seizures, tonic-clonic seizures, atonic seizures, and myoclonic seizures. They occur suddenly without auras or other focal symptoms.
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Localization-related epilepsy
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Recurrent focal seizures that arise from a single unilateral brain region or multiple discrete areas in the brain. Idiopathic causes have a known genetic basis and include AD benign rolandic epilepsy and temporal lobe epilepsy, whereas symptomatic causes involve acquired pathologies such as strokes, neoplasms and congenital malformations. Cryptogenic causes are associated with clinical, mental and developmental retardation with no obvious structural lesion.
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Simple partial seizure
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Focal seizure with no impairment of consciousness. Auras such as odd smell or taste are simple partial seizures.
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Complex partial seizure
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Focal seizure with impairment of consciousness during or after the event. Both simple and complex partial seizures can spread to produce secondary generalized tonic-clonic seizures with urinary incontinence, tongue-biting, and postictal confusion.
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Status epilepticus
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Neurologic emergency in which a seizure persists of recurs for 30 minutes without return to baseline mentation. Some authorities believe that seizure activity is unlikely to stop after 5 -10 minutes and should be treated sooner to avoid injury.
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Muscular dystrophy
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Inherited disease characterized by progressive weakness and degeneration of the skeletal muscles that control movement.
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Dystrophin protein
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Rod-shaped protein, and a vital part of a protein complex that connects that cytoskeleton of a muscle fiber to the surrounding extracellular matrix through the cell membrane.
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Tourette syndrome
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Tics: brief and episodic movements or sounds induced by internal stimuli that are only temporary suppressible.
Autistic spectrum disorders: impaired social interactions, poorly developed language, and frequent cognitive impairment. Obsessions and compulsions: obsessions are intense and often intrusive thoughts, which compel patients to perform mostly meaningless, time consuming and sometimes embarrassing rituals or compulsions. |
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Lissencephaly
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Smooth brain genetic malformation of the cerebral cortex in which abnormal neuronal migration during early development results in smooth cerebral surfaces with absent (agyria) or decreased (pachygyria) convolutions.
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Miller-dieker syndrome
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Severe lissencephaly phenotype secondary to deletion on chromosome 17p13.3 with agyria and characteristic dysmorphic features
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Isolated lissencephaly sequence
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Milder phenotype compared to dieker syndrome with pachygyria and mild or absent dysmorphic features because of autosomal dominant mutations in the LIS 1 gene on chromosome 17p13.3 or X-linked mutation sin the doublecortin (DCX) gene on chromosome Xq22.3. pachygyria caused by LIS 1 mutations is posterior-predominant on neuroimaging whereas anterior-predominant pachygyria is more typical of DCX mutations.
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Infantile spasms
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Dramatic repetitive bouts of rapid neck flexion, arm extension, hip and knee flexion, and abdominal flexion, often with arousal from sleep. The mother might describe them as unprovoked startle responses or colicky spells as a result of abdominal pain, although there is not crying typical of colic. Typical presentation occurs between 3 to 8 months.
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Hypertelorism
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Abnormally increased distance between the eyes.
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Epicanthal fold
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Skin fold of the upper eyelid (from the nose to the medial side of the eyebrow) covering the medial corner (medial canthus) of the eye
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Clinodactyly
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Congenital condition where the little finger is curved toward the ring finger
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Opisthotonus
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Severe hyperextension of the back caused by spasm of the muscle along the spinal column.
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Auditory brainstem response
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An electrical evoked hearing test. Electrodes are placed on each earl lobe and on the forehead. A stimulus sound is delivered into the test ear at a specified loudness: an attached computer captures the electrical brain activity results from this stimulus and filters out background noise.
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Cerebellopontine angle
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Anatomic space between the cerebellum, pons and temporal bone. Space contains cranial nerves V through XI.
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Conductive hearing loss
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A form of hearing loss that results from a defect in the sound collecting mechanism of the ear. (ear canal, tympanic membrane, middle ear and the ossicles)
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Epidermoid tumor
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A benign tumor composed of squamous epithelial elements thought to arise from congential rests
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Glomus tumor
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Common name for paraganglioma. Highly vascular tumor arises from neuroepithelial cells. Tumors are further named by structures that they arise from:
Glomus tympanicum (middle ear) Glomus jugulare (jugular vein) Glomus vagale (vagus nerve) Carotid body tumor (carotid artery) A rule of 10% associated: ~ 10% of these tumors produce a catecholamine-like substance, ~10% of these tumors are bilateral, ~ 10% are familial are malignant. |
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Meningioma
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Common benign extra-axial tumors of the coverings of the brain. The cell or origin is probably from arachnoid villi. Several histologic subtypes are described: syncytial, transitional, fibroblastic, angioblastic and malignant.
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Sensorineural hearing loss
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A form of hearing loss that results from an abnormality in the cochlea or auditory nerve.
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