Lecture 9 (neuro)-Exam 3 Flashcards

Question

First Unprovoked Seizure Treatment * Generally accepted principle that when a patient with a first seizure suffers 1 or more ensuing seizures, what should be initiated? * For adults with unprovoked first seizure: immediate what needs to be done?

Answer 1

* Generally accepted principle that when a patient with a first seizure suffers 1 or more ensuing seizures, an AED should be initiated because the risk for, yet additional seizures is very high (57% by 1 year and 73% by 2 years) * For adults with unprovoked first seizure: immediate AED reduce risk of seizure recurrence to approx. 35% over the next 2 years

Answer 2

**Fingerstick glucose testing – if proven hypoglycemia, IV dextrose should be provided.** First line - IV Benzodiazepam (Lorazepam) initially Initial treatment failure occurs due to: * Using inadequate initial doses due to concern for respiratory compromise * Waiting too long to repeat benzodiazepam doses and advancing to second line agents

Answer 3

If after 20 minutes they continue, two adequate doses of benzodiazepines and no correctable underlying etiology is found then move to SECOND LINE treatment. Second line treatment: * Levetiracetam (Keppra) * Phenytoin (Dilatin) * Valproate sodium (Depakene) – used if patient has allergy to phenytoin

Answer 4

Management is focused on three main goals: * controlling seizures * avoiding treatment side effects * maintaining or restoring quality of life Clinicians should assist in empowering patients with epilepsy to lead lifestyles consistent with their capabilities.

Answer 5

* Initial treatment is monotherapy * Consideration is based upon combination of drug, seizure, patient specific factors (gender, age, cost, availability of the medications)

Answer 6

* considering drug interaction limitations that may make the seizure medication less effective * hepatic and renal diseases impact the choice of seizure therapies (metabolized by the liver or excreted by the kidneys) * women in childbearing age: teratogenic effects of seizure medications * monitor mood and suicidality with AEDs

Answer 7

Counseling patients about their risks for seizure recurrence and options for management. * 70% of patients with proper diagnosis and treatment of epilepsy could live seizure free Emphasize the importance of compliance with seizure medications Discuss driving and operating machinery

Answer 8

previous head injury → chronic subdural hematoma limb twitch/ incontinence → epilepsy sudden collapse → hemorrhage * Intracerebral Hemorrhage (ICH) * Subarachnoid Hemorrhage (SAH)

Answer 9

* Mass lesion → Malignancy * Diabetes → Hypo / Hyperglycemia * Alcoholism/ Drug Abuse/Psychiatric illness → Drug overdose/toxicity * Viral Infection → Encephalitis

Answer 10

* Multiple sclerosis is the most common immune mediated inflammatory demyelinating disease of the central nervous system. * Inflammation and demyelination primarily involves the white matter of the spinal cord.

Answer 11

* **Patients with MS typically follow either a relapsing-remitting pattern of episodes or a primary progressive course.** * Damage is random and asymmetric. * A viral infection may act as a precipitant.

Answer 12

* Disease often begin between 20 and 30 years of age. * Disease is most common in women.

Answer 13

* Etiology is unknown, immune or viral factors are suspected but unproven causes. * Thought to be multifactorial in origin, with both environmental and genetic factors play a role.

Answer 14

* **Onset of MS symptoms usually occurs over several days and is seldom sudden**. * Initial symptoms often relate to motor dysfunction. * **Weakness in the legs, and less commonly, their arms.** * **May complain of foot drop, causing difficulty walking, or causing them to trip on sidewalks or curbs.**

Answer 15

* Weakness and stiffness in MS can reflect compromise of the corticospinal tract. * Also have sensory symptoms, such as tingling, pins and needle sensation, numbness, or a sensation of a band around the torso. * **May also complain of sensations of electricity running down their spine, sometimes extending down into their limbs, aka Lhermitte sign.**

Answer 16

* Clinically isolated syndrome — patient with a first clinical attack, difficult to predict whether a given individual will develop MS following symptom onset. It places the patient at risk for further relapses. * **Relapsing-remitting MS: the most common type, characterized by sudden onset of neurological symptoms that usually last several weeks and then resolve, often leaving few or no deficits.**

Answer 17

* Secondary Progressive Syndrome- gradual worsening of an initially remitting relapsing syndrome. Fewer and less complete remissions after each attack. * **Primary progressive MS —insidious neurological progression of neurological disabilities suggestive of primary progressive MS (no acute relapses)**

Answer 18

One or more hyperintense T2 (type of MRI image) lesions characteristic of MS in one or more of the periventricular, cortical, or infratentorial areas * Areas of demyelination in the brain/spinal cord that appear on T2 MRI sequence films Two or more hyperintense T2 lesions in the spinal cord.

Answer 19

If a patient has symptoms reflecting two or more separate brain lesions over TIME, and this is confirmed on MRI brain imaging, SPACE, then MS should be suspected

Answer 20

“Space and Time" criteria ensure that the diagnosis of MS is based on a pattern of disease activity over time and affecting multiple parts of the CNS, rather than a single event or lesion. This helps distinguish MS from other neurological disorders and confirms the chronic, progressive nature of the disease.

Answer 21

**Presence of CSF-specific oligoclonal bands/Myelin Basic Protein** * Oligoclonal bands are present in up to 90% of MS patients. **Dawson Fingers** if present confirm diagnosis * Ovoid lesions extending from lateral ventricles into deep cerebral white matter

Answer 22

Autoantibody testing — Testing for the aquaporin-4 (AQP4) IgG serum autoantibody and the myelin oligodendrocyte glycoprotein IgG autoantibody (MOG-IgG) are indicated for patients presenting with acute central nervous system demyelination when clinical, imaging, or laboratory features are atypical of MS.

Answer 23

Inflammation in conjunction with blood-brain-barrier disruption, characterized by gadolinium enhancement on MRI, is seen in the early stages of most demyelinating lesions in patients. * lesions are bright on MRI and seen in the corpus callosum and periventricular regions.

Answer 24

* Inflammatory T cells, B cells, and macrophages are typically seen on histopathologic samples * **The presence of immunoglobulin G (IgG) and immunoglobulin M (IgM) and oligoclonal bands in the cerebrospinal fluid (CSF)**. * MS is a clinical diagnosis that can be supported mainly by MRI of the brain. CSF investigations can be supportive.

Answer 25

Corticosteroids may assist maximal recovery from acute exacerbations. High dose IV corticosteroids often are used in the setting of optic neuritis. * has not been shown to decrease the risk of future attacks or change the course of the disease but are for treating current symptoms

Answer 26

Plasma exchange can give benefit to patients with acute exacerbations who do not respond to high dose IV corticosteroids. * must be used on a chronic, ongoing basis. Symptomatic therapy for MS can include physical, emotional rest, physical therapy as well.

Answer 27

Methyl-prednisolone (Solumedrol): High doses Oral Prednisone with or without a taper. ACTH Adrenocorticotropic Hormone IM administration: * stimulates the adrenal glands to produce corticosteroids which have potent anti-inflammatory properties Plasma exchange treatments for patients with severe neurological deficits

Answer 28

* Cranial Nerves * Motor Testing * Sensory Testing * Reflexes * Gait and Coordination * Skin Examination

Answer 29

* A chronic impairment of muscle tone, strength, coordination, or movements. **It is believed to result from cerebral injury before birth, during delivery, or in the perinatal period**. * Clinical features are widely varied and include spasticity, lack of coordination, fine motor difficulties, ataxia, lethargy, hypotonia or dystonia. **Spasticity being the most prominent**.

Answer 30

Physical exam reveals hyperreflexia, hypotonia, microcephaly, limb length discrepancies, cataracts and congenital heart disease.

Answer 31

* One of the first signs that may lead to suspicion of CP is a delay in reaching developmental milestones. This could include delays in rolling over, sitting, standing, or walking. Parents or pediatricians may notice these delays during routine check-ups, which can prompt further investigation. * MRI and genetic testing is required to exclude other conditions

Answer 32

* Treatment is supportive, with the goal of attaining maximum function and potential in physical, occupational, and speech ability. * Pharmacological treatment of spasticity and seizures often required.

Answer 33

Myopathy refers to a clinical disorder of the skeletal muscles. Abnormalities of muscle cell structure and metabolism that leads to various patterns of weakness and dysfunction.

Answer 34

* Cardiomyopathy: involve cardiac muscle fibers, resulting in a hypertrophic or dilated cardiomyopathy * Inflammatory/ autoimmune myopathy: Characterized by inflammation of muscles and peri-muscular space (Polymyositis, dermatomyositis, sarcoidosis, lupus, and rheumatoid arthritis)

Answer 35

* Toxic myopathy: This occurs when a toxin (ETOH, Cocaine. Medications (Antimalaria Chloroquine, Statins, Antipsychotic, Chemotherapy agents..) impair muscle structure and function. * Endocrine myopathy: Hormones interferes with muscle function such as Hypo/Hyperthyroid, Diabetes, Cushing’s syndrome.

Answer 36

* Infectious myopathy: Viral Myositis, HIV, Hepatitis… Necrotizing fasciitis, Polymicrobial related to aerobic and anaerobic bacteria, monomicrobial like Staph. Aureus and hemolytic strep. * Myopathy secondary to electrolyte imbalance: Electrolyte problems, such as excessively high or low potassium levels ( Hypokalemic Periodic Paralysis) Hypoglycemia, Hypocalcemia, Hypomagnesemia

Answer 37

* Hereditary/ Congenital: Muscular dystrophies: Limb Girdle muscular dystrophy, muscular dystrophy, Myotonic dystrophy, Occulopharyngeal muscular dystrophy * Traumatic Rhabdomyolysis: breakdown of skeletal muscle tissue due to physical trauma.

Answer 38

* **Muscle weakness, impaired function in activities of daily life, muscle pain and tenderness**. * **Symmetric proximal muscle weakness** * Malaise, fatigue * Absence of sensory complaints or paresthesia * **Deep tendon reflexes (DTRs) may be diminished/absent in hypokalemic paralysis** * Very late findings: Atrophy and hyporeflexia

Answer 39

* Laboratory Studies – chemistry and urinalysis, elevation of plasma muscle enzymes, Creatinine Kinase (CK), Lactic Dehydrogenase, RBC in the urine is suggestive of myoglobinuria. * Serologic Tests – ANA, Antibodies against extractable antinuclear antigen, Antineutrophil Cytoplasmic Antibody (ANCA) for vasculitis and other autoimmune diseases.

Answer 40

* Electrophysiologic Studies – Nerve conduction and Electromyographic (EMG) Studies * Muscle Biopsy * Genetic Testing for patients with possible heritable myopathies and muscular dystrophies.

Answer 41

* Treatment of myopathies is multidisciplinary and depends on the type of myopathy. * Certain types of myopathies can be treated with steroid, immune suppressing therapy and IVIG.

Answer 42

* Most myopathies require the use of supportive services, such as physical and occupational therapy, pulmonary medicine, cardiology, dietary management, and speech/swallowing therapists. * Surgical treatment of spine and limb deformities is used in long-standing cases.

Answer 43

* Myasthenia gravis is an autoimmune disease where T-cells, B-cells produce antibodies which block and destroy Acetyl-Choline receptors faster than new ACh receptors can be synthesized. * Proximal weakness is weakness predominantly affecting the axial muscles, deltoid and hip flexors.

Answer 44

* Weakness quickly fluctuates and worsens throughout the day. * Fatigability of the muscles with recovery to the baseline strength after a short period of rest.

Answer 45

* Will develop ocular symptoms – ptosis can be bilateral or unilateral and can shift quickly from one eye to the other. * **Limb weakness is most common** – presents as having difficulty raising arms above the head, having difficulty climbing up the stairs, and rising from a chair. * **Key clinical exam finding is fatigability causing increased weakness with use of the muscle.**

Answer 46

Clinical diagnosis is established by history and exam. * reflexes and sensation are NORMAL

Answer 47

Clinical test in patients with ptosis is the ice test – ice is placed over the eyelid for 2 minutes – if ptosis improves after removing the ice, diagnoses can be made. * cooling improves neuromuscular junction transmission, heat worsens it. * hence Myasthenia Gravis is worse in the summer

Answer 48

Serologic tests for autoantibodies, Ach receptor antibodies * three antibodies – binding, blocking and modulating * need a positive test for one of these antibodies

Answer 49

Electrophysiologic studies such as repetitive nerve stimulation and single fiber EMG studies. CT or MRI of the chest/mediastinum looking for Thymoma (75% will have it) * Tumor of the thyroid

Answer 50

Tensilon Test – agent given which temporarily inhibits the enzyme Ach – with more present, makes more acetylcholine available to stimulate the receptors on muscle cells and temporarily improve muscle strength in MS.

Answer 51

Myasthenia Gravis is a chronic but treatable disease, most patients can achieve sustained remission of symptoms and full functional recovery.

Answer 52

* Pyridostigmine: oral acetylcholinesterase inhibitor as initial therapy for mild to moderate Myasthenia Gravis – only treats symptoms. * Glucocorticoids: Prednisone is used. * Immunosuppressive therapy: immunosuppressive agents are the mainstay for treatment. IVIG, Plasma Exchange for myasthenia crisis

Answer 53

* Is a disorder of reduced acetylcholine release from the presynaptic nerve terminals. * 50% of cases are associated with malignancy, **mainly small cell lung cancer**. * LEMS can be a paraneoplastic syndrome – disorder that occurs because of an immune system response to a cancerous tumor elsewhere in the body

Answer 54

It is associated with slowly progressive proximal muscle weakness mostly in the legs; deep tendon reflexes are typically depressed or absent * lower limb weakness > upper limb weakness

Answer 55

* It is associated with dry mouth and other autonomic symptoms. * **Key clinical finding is that a patient may regain strength with repeated activation of muscles.** * Tx with Firdapse (Amifamridine)-> If associated with tumor, LEMS may go away with tumor treatment

Answer 56

* Mononeuropathy: focal involvement of a single nerve due to trauma or compression or entrapment (carpal tunnel syndrome, CTS) * Mononeuritis Multiplex: simultaneous or sequential involvement of non-contiguous nerve trunks. Compression neuropathies, systemic vasculitis.

Answer 57

* Peripheral Neuropathy: Disorder of the peripheral nervous system including radiculopathies and mononeuropathies. * Radiculopathies: Cervical, thoracic and lumbar

Answer 58

* **Gillian Barre Syndrome: Acute Polyradiculoneuropathy disorder** * Complex Regional Pain Syndrome: previously called(Reflex Sympathetic Dystrophy, RSD)

Answer 59

Chronic axonal polyneuropathies which can be due to diabetes or uremia are the most common of polyneuropathies’.

Answer 60

* Acute axonal polyneuropathy: affects multiple peripheral nerves and is characterized by rapid onset and damage to axons, the long thread-like parts of nerve cells. * Chronic Inflammatory demyelinating polyneuropathy were weakness and numbness often present simultaneously.

Answer 61

Gillian-Barre syndrome which starts predominantly in motor nerve fiber with weakness rather sensory loss, typically the earliest signs of the disease, then dysesthesia in arms or legs with gait difficulty.

Answer 62

* Symmetric distal sensory loss * Burning sensation * Weakness is distal * Walking difficulties /balance problem * Slowly progressive presentation of sensory loss, chronic pain with numbness and burning sensation and pain in the feet with mild gait abnormality this progress is to mild weakness of the lower legs and hand symptoms the resulting in the classic glove and stocking distribution of sensory loss

Answer 63

* In addition to the history of neuropathy itself the patient should be asked about recent viral illnesses, systemic illnesses, new medications, exposure to solvents or heavy metals or other potential toxins such as alcohol use and family history of neurologic disease. * Diagnostic evaluation with atypical features as asymmetry, motor predominance, acute onset prominent autonomic involvement, severe or rapidly progressive symptoms, and sensory ataxia.

Answer 64

* **Electrodiagnostic testing with EMG and or nerve conduction study as the initial diagnostic procedure for patient with suspected polyneuropathy when there is no clear etiology/atypical features.** * **Laboratory testing : blood glucose, serum B12 level, with and without Homocysteine, serum protein electrophoresis SPEP and thyroid function tests.** * Nerve biopsy is useful for vasculitis or amyloidosis

Answer 65

Treatment with the underlying disease * tight control of blood sugar to maintain nerve function * thyroid replacement therapy for hypothyroidism * eliminating toxins and treating vasculitis which is inflammation of the blood vessels

Answer 66

Alleviation of symptoms related to the illness: to reduce pain associated such as with Pregabalin, tricyclic antidepressants.

Answer 67

* Acute illness causing a rapidly progressive polyneuropathy with weakness or paralysis. * Cardinal signs for GBS are progressive, mostly symmetric ascending muscle weakness with depressed or absent tendon reflexes (90%)

Answer 68

* Primarily affects motor roots and produces lower motor neuron symptoms – muscle weakness, flaccid paralysis, and hyporeflexia. * Upper cervical root involvement and respiratory paralysis are common. * Sensory fibers are affected, resulting in paresthesia. * GBS usually progresses over a period of two weeks.

Answer 69

* GBS is thought to be the result of immune response to a preceding infection which cross-reacts with peripheral nerves, such as acute influenza. * Symptoms develop 1-4 weeks following infection

Answer 70

The immune response can be directed towards the myelin or axon of peripheral nerves resulting in demyelinating and axonal form of GBS. * **Campylobactor jejuni infection is the most common cause of GBS**

Answer 71

* CMV, EBV, HIV, Zika virus have been associated with GBS * Immunization, Trauma, surgery may trigger GBS

Answer 72

* The typical finding with lumbar puncture is elevated cerebrospinal fluid protein with normal white blood cells. * Electrodiagnostic study such as EMG and nerve conduction study may show evidence of acute polyneuropathy with predominantly demyelinating features or predominantly axonal features in acute motor or sensory neuropathies. * GQ1B antibody associated with Miller Fisher variant of GBS

Answer 73

* **Supportive care is extremely important in GBS 30% of patients who develop neuromuscular respiratory failure requiring mechanical ventilation.** * Autonomic dysfunction warrant ICU monitoring

Answer 74

* Cardiac and blood pressure monitoring * Abdominal auscultation to monitor for ileus * Disease modifying therapies are plasma exchange and IVIG intravenous immunoglobulin

Answer 75

ALS is a relentlessly progressive, presently incurable, neurodegenerative disorder that causes muscle weakness, disability, and eventually death based on the involvement of upper and lower motor neurons. * Example would be progressive upper extremity weakness, spasms in calves, muscle twitching. ## Footnote Referred to as Lou Gehrig’s Disease

Answer 76

Muscle weakness & atrophy in hands is usually the 1st sign

Answer 77

ALS is characterized by motor neuron degeneration and death with gliosis (reactive changes) replacing lost neurons. * Sensory, proprioception and coordination may be preserved Cortical motor cells (pyramidal and Betz cells) in the motor cortex, anterior horn cells of the spinal cord and lower cranial motor nerves in the brainstem breakdown and die.

Answer 78

This gliosis results in the bilateral white matter changes sometimes seen in the brain magnetic resonance imaging (MRI) of patients with ALS. * MRI of the brain and cervical spinal cord may be unremarkable The spinal cord becomes atrophic.

Answer 79

ALS is the most common form of motor neuron disease. * Hoffman sign

Answer 80

* Evidence of upper motor neuron and lower motor neuron signs and spare sensory neurons. * Progression of disease (no relapsing or remitting), and the absence of an alternative explanation. * Treatment is supportive

Answer 81

Tremor: rhythmic oscillating movement across a joint, representing contraction of agonist & antagonist muscles

Answer 82

* Rest tremor: may involve arms, legs, or jaw (e.g. Parkinson’s disease) * Postural tremor: maximal when patient holds extremity suspended against gravity (e.g. Essential Tremor) (stretched out hands) * Action tremor: maximal during movement of a limb (e.g. Cerebellar Tremor) finger to nose

Answer 83

* Chorea: A state of excessive, spontaneous movements that are non-repetitive of moderate amplitude & speed with a flowing “dance-like” quality * Dystonia: Sustained abnormal muscle contraction producing pain &/or a positional deformity (torticollis) * Myoclonus: Lightening-like muscle jerks that may involve any body part, often repetitive * Tic: Abnormal repetitive stereotypic movements (motor) or sounds (vocal) that usually begin in childhood or adolescence.

Answer 84

* One can make a diagnosis with 90% certainty simply by watching the tremor. * If the tremor is slow, it is either a cerebellar tremor, which becomes worse with intention, or a parkinsonian tremor, which is worse at rest. * If the tremor is rapid, it is primary, chemical.

Answer 85

Most common cause of tremor. Slowly progressive disorder * Autosomal dominant inheritance – primary Postural tremor affecting hands most often.

Answer 86

Impaired job performance and embarrassment. Exacerbated by anxiety, emotional stress and caffeine.

Answer 87

Diagnosis based on history and physical If the tremor appears when holding a posture, then it is essential tremor Labs: thyroid function tests – r/o hyperthyroidism

Answer 88

Treatment: * Propranolol (Inderal) – sexual and depressive side effects * Deep brain stimulation * Botox into muscle that are responsible of tremor Most striking and diagnostic feature of essential tremor is said to be the dramatic relief with alcohol.

Answer 89

* Parkinsonism is a clinical syndrome presenting with any combination of bradykinesia, rest tremor, rigidity, and postural instability. * The most common form is Parkinson disease (PD), a chronic, progressive disorder caused by degenerative loss of dopaminergic neurons in the brain.

Answer 90

* PD remains a clinical diagnosis, based on the clinician's ability to recognize its characteristic signs and associated symptoms, especially in the early stages. * Parkinson disease is thought of as a motor disorder, sensory systems are also affected. Loss of sense of smell is almost universal.

Answer 91

* Bradykinesia is defined as slowness of movement plus a decrease in amplitude/speed or progressive hesitations/halts as movements are continued. * Rigidity is a velocity-independent resistance (sometimes referred to as “cogwheel” or "lead-pipe resistance") to passive movement of the major joints while the patient is in a relaxed position.

Answer 92

Resting tremor is a 4 to 6 Hz tremor that is observed in a fully resting limb and is suppressed when initiating movement (pill-rolling). * Is an early manifestation of Parkinson disease Other signs: Masked faces (Hypomimia), Decreased Blink rate, Speech impairment (hypophonia). Patients with Parkinson disease will have difficulty performing two tasks as the same time - "tap the top of your head and stick out your tongue to me"

Answer 93

* Early onset of, or rapidly progressing, dementia * Rapidly progressive course * Upper motor neuron signs * Cerebellar signs – ataxia * Urinary incontinence * Early symptomatic postural hypotension * Early falls * If the tremor is persistent only at rest, this almost guarantees the diagnosis. | Majority of cases of Parkinson disease are unknown.

Answer 94

Treatment is initiated when the patient’s quality of life is affected and usually consists of either: * **Carbidopa/Levodopa can help to alleviate the symptoms**. No treatment affects the degenerative process, symptomatic treatment is the mainstay of therapy. Physical measures such as physical therapy, speech therapy, and exercise are important and have a major impact on their lives

Answer 95

AKA Willis-Ekbom disease which affects up to 15% of population Genetic predisposition has been described and occurs earlier in life * related to disturbance of dopaminergic receptors and diminished brain iron stores

Answer 96

Chronic movement disorder in which patients have an irresistible urge to move their legs * there is usually no pain, and urge is relieved by movement * symptoms are usually worse at night causing sleep disturbance

Answer 97

* sensations such as crawling, creeping, pulling, itching, drawing, or stretching, all localized to deep structures rather than the skin * periodic leg movements (foot dorsiflexion) lasting 1-5 seconds and occurring every 20-40 seconds during sleep

Answer 98

Diagnosis: exam is usually normal, therefore historical points are essential * EMG/blood work can be done to r/o other causes * Polysomnography can quantify the frequency of leg movements * iron studies should be done (typically low)

Answer 99

* vibration devices for feet/lower legs * avoid caffeine, dopamine blocking agents and antidepressants and replace iron * medications reserved for moderate symptoms (dopamine agonists)

Answer 100

Involuntary and uncontrolled movement disorders stemming from dopamine receptor blockade (medication induced) ## Footnote "Tardive – means late or after a long period of treatment"

Answer 101

Feeling of restlessness, accompanied by a constant desire to move. Involuntary muscle contractions such as eye spasms, twisting of the head. Typically, in patients who are chronically on typical antipsychotics (schizophrenia/bipolar) * symptoms may persist even if medications are stopped

Answer 102

TD symptoms improve with movement (like parkinsonism) Abnormal Involuntary Movement Scale (AIMS) * baseline should be obtained prior to initiation of medication treatment Get a CT or MRI to r/o other causes as they are usually normal in drug induced TD

Answer 103

avoid dopamine antagonists * continued use of these maintain an increased or heightened state of dopamine receptors. * persistent blockage and resulting neurochemical imbalance can make symptoms of TD worse * Valbenazine approved in 2017 * Benztropine (Cogentin) has been used off-label

Answer 104

* Age of onset ranges from childhood to the eighth decade but is most common in mid-life. * Autosomal dominant disorder, characterized by psychiatric disease.

Answer 105

* Three major symptoms – involuntary movements, changes in behavior and personality, and cognitive impairments. * Variable flowing movements of the limbs and trunk, reflexes that are brisk, gait that is rapid, wide based and uncoordinated. * Chorea is a major manifestation of Huntington's Disease.

Answer 106

The pathophysiology of HD is not fully understood, although it is thought to be related to toxicity of the mutant huntington protein involving putamen and caudate atrophy. No known cure, treatment is symptomatic and remains supportive.

Lecture 9 (neuro)-Exam 3 Flashcards

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