Neuro Disorders

Question 1

Amyotrophic lateral sclerosis

Answer 1

ALS is a progressive motor neuron disease characterized by dysfunction of both upper motor neurons (UMNs) and lower motor neurons (LMNs) in the corticospinal and corticobulbar tracts, anterior motor horn cells, and bulbar motor nuclei

Question 2

ALS Onset

Answer 2

55-75 years old
Few risk factors
Familial history may be a risk

Question 3

ALS Presentation

Answer 3

Precise documentation of history of symptoms and exam is key
Early Lower Motor Neuron death = asymmetric weakness in limbs, foot drop, difficulty moving limbs
Early Upper Motor dysfunction = hypperreflexia, spasticity, Babinski sign, weakness, fasiculations
Bulbar Dysfunction = dysarthria, dysphagia, tongue atrophy, tongue irregular movement

Question 4

ALS Diagnosis

Answer 4

diagnosis of ALS is usually made when there are widespread UMN and LMN signs in the absence of any electrophysiologic and pathologic signs of other disease processes as well as absence of neuroimaging evidence of other disease processes

Rule out heavy metal exposure, thyroid, CSF causes, electrolyte / vitamin issues, nerve conduction for parkinson

Question 5

ALS Managment

Answer 5

Symptom Management
Riluzole - antiglutimate that improves survival, slows progression but liver damaging
Edaravone - reduces oxidative stress, infusion then injections, used as adjunct
SSRI/SNRI for dperession
Benzo to manage anxiety
Tizanidine and baclofen for spasticity

Poor life expectancy

Question 6

Bell Palsy

Answer 6

acute, unilateral weakness or paralysis of the facial nerve, with an onset of less than 72 hours and unknown etiology, Bell palsy is the most commonly diagnosed peripheral facial nerve condition

Question 7

Bell Palsy Presentation

Answer 7

Acute and progressive pain behind ear then facial paralysis
-smooth forehead, inability to close eye, asymmetric smile, tearing, drooling, tinnitus
History of recent infection or viral illness

Question 8

Bell Palsy Diagnosis

Answer 8

routine diagnostic tests and imaging are not recommended for new-onset Bell palsy.

Rule out CVA/TIA and Lyme Disease

Refer if patient is pregnant, corneal abrasion, no improvement in 2 weeks, atypical presentation

Question 9

Bell Palsy Treatment

Answer 9

Protection of the eye is critical

Corticosteroids within 72 hours of symptoms

Question 10

Ischemic Stroke

Answer 10

In ischemic stroke, the patient usually has a single attack, and the entire event evolves within a few hours. However, the stroke may occur in a “stuttering” fashion, with intermittent progression or fluctuation of neurologic deficits that extends to maximal deficit over the first 72 hours

The classic visual disturbance (amaurosis fugax) is a transient, painless loss of vision, often described as a shade descending over the visual field.

Question 11

Subarachnoid Hemorrhage

Answer 11

the clinical presentation is usually heralded by the abrupt onset of a severe headache (“the worst headache of my life”), nausea and vomiting, signs of meningeal irritation, and varying degrees of neurologic dysfunction. Loss of consciousness at the time of the initial event is common but is usually short-lived. Nearly 50% of patients with aneurysmal SAH give a history of atypical headaches occurring days to weeks before the definitive event.

Question 12

Intracerebral Hemorrghage

Answer 12

no consistent warning or prodromal symptoms. In the majority of cases, the hemorrhage has its onset while the patient is up and active; onset during sleep is rare. The blood pressure is elevated in almost all cases. The neurologic signs and symptoms vary with the site and size of the extravasation of blood. The patient may lapse almost immediately into stupor and coma, with hemiplegia and steady deterioration to death during the next several hours. More often, the patient complains of a headache, followed within a few minutes by unilateral facial sag, slurred speech, weakness in an arm and leg, and eye deviation away from the paretic limbs. These events, occurring during a period of 5 to 30 minutes, strongly suggest intracerebral bleeding.

Question 13

Dementia

Answer 13

dementia comprises several symptoms, including a progressive loss of memory and behavioral changes, which together interfere with independence in activities of daily living

Question 14

Mild Cognitive Impairment

Answer 14

MCI is thought to be a transitional state between normal aging and dementia. Because individuals with MCI may progress to dementia at a rate of 10% to 15% a year, MCI is considered a risk factor for all types of dementia, and these patients need close monitoring and follow-up

Question 15

Alzheimer’s Disease

Answer 15

Alzheimer’s disease is characterized by amyloid plaques and neurofibrillary tangles. Examinations of the brains of patients with Alzheimer’s disease show atrophy of the cerebral cortex that is usually diffuse but may be more pronounced in the frontal, temporal, and parietal lobes

Question 16

Vascular Dementia

Answer 16

Multiple areas of focal ischemic change characterize vascular dementia, formerly known as multi-infarct dementia. The defining lesion is the lacunar infarct. Lacunae are defined as gaps, missing areas, or holes

Question 17

Lewy Body Dementia

Answer 17

Lewy body dementia is characterized by the presence of Lewy bodies in the brain. These are proteins that enter neurons and cause cell degeneration and death. There is a loss of dopamine-producing neurons, similar to that seen in Parkinson’s disease, and a loss of acetylcholine, similar to that seen in Alzheimer’s disease

Question 18

Dementia Presentation

Answer 18

Subtle, chronic memory loss, personality changes, loss of daily activity ability, impaired cognition
Usually a family member makes the initial presentation

Lewy Body may also have visual hallucinations, motor impairment, and haloperidol will worsen agitation

Question 19

Alzheimer’s Stages

Answer 19

Initial is memory loss
Early stage = anxiety and depression
Second stage = worsening of memory and language, impaired judgement, disorientation
Final stage = motor rigidity, neuro dysfunction, severe cognitive dysfunction

Question 20

Dementia Diagnosis

Answer 20

dementia has no single standard test and is a disease of exclusion, the diagnostic evaluation should determine whether the patient has a reversible condition that may be contributing to or causing cognitive decline. The most important tests include a complete blood count (CBC), thyroid-stimulating hormone (TSH) concentration, vitamin B12 and folate levels, and a metabolic screen. Check medication levels to rule out medication effects.

Question 21

Delirium

Answer 21

although dementia and delirium both include global cognitive impairment, delirium is characterized by prominent deficits in attention and awareness of the environment, and the symptoms typically develop rapidly and fluctuate in severity.

Question 22

Delirium in Elderly

Answer 22

often the first and only indicator in older adults of underlying physical illness, such as infection, myocardial infarction, or drug toxicity; it is the leading complication of hospitalization for older adults

May occur when patient has dementia (delirium superimposed on dementia) - there is acute change in their baseline

Question 23

Pseudodementia

Answer 23

Depression in older adults can lead to memory loss, attention deficits, and problems with initiation, and is referred to as “pseudodementia.”

Question 24

Dementia Management

Answer 24

Vitamin E may help, but not if on anticoagulants
Donepezil, Rivastigmine, Galantamine (cholinesterase inhibitors)
Memantine is NMDA antagonist used to help slow progression
Citalopram may help agitation

Question 25

Which diagnostic test helps confirm a diagnosis of Guillain–Barré syndrome in a patient who is developing muscle weakness and paresthesias?

Answer 25

A lumbar puncture is the most important confirmatory test showing albuminocytologic disassociation.

Question 26

Guillain-Barré syndrome (GBS)

Answer 26

group of acute monophasic immune-mediated peripheral neuropathies

Two-thirds of the time, GBS follows an upper respiratory or gastrointestinal infection by 1 to 4 weeks. The most commonly identified viruses are cytomegalovirus (CMV) and Epstein-Barr virus, and bacteria include Campylobacter jejuni

Question 27

GBS Symptoms

Answer 27

onset of relatively symmetric paresthesias and/or weakness, typically starting in the lower extremities and evolving over hours to days. Frequently there is a history of an antecedent infection. They often have back pain but do not have bowel or bladder dysfunction early in the course.

Progressive weakness, beginning with the legs and progressing to the arms with an evolving loss of deep tendon reflexes, is typical of GBS

Question 28

GBS Diagnostics

Answer 28

Lumbar puncture is best confirmatory test (elevated CSF protein without elevated CSF WBCs)
Screening for antecedal infection is crucial
Often SIADH occurs

Rule out spinal cause, toxins, other neuro cause

Question 29

GBS Management

Answer 29

Hospitalization to monitor progressive weakness, provide supportive care, and manage potential complications is necessary in all but the mildest suspected cases of GBS.