Neuro Disorders Flashcards
Amyotrophic lateral sclerosis
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
ALS Onset
55-75 years old
Few risk factors
Familial history may be a risk
ALS Presentation
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
ALS Diagnosis
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
ALS Managment
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
Bell Palsy
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
Bell Palsy Presentation
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
Bell Palsy Diagnosis
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
Bell Palsy Treatment
Protection of the eye is critical
Corticosteroids within 72 hours of symptoms
Ischemic Stroke
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.
Subarachnoid Hemorrhage
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.
Intracerebral Hemorrghage
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.
Dementia
dementia comprises several symptoms, including a progressive loss of memory and behavioral changes, which together interfere with independence in activities of daily living
Mild Cognitive Impairment
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
Alzheimer’s Disease
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
Vascular Dementia
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
Lewy Body Dementia
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
Dementia Presentation
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
Alzheimer’s Stages
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
Dementia Diagnosis
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.
Delirium
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.
Delirium in Elderly
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
Pseudodementia
Depression in older adults can lead to memory loss, attention deficits, and problems with initiation, and is referred to as “pseudodementia.”
Dementia Management
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
Which diagnostic test helps confirm a diagnosis of Guillain–Barré syndrome in a patient who is developing muscle weakness and paresthesias?
A lumbar puncture is the most important confirmatory test showing albuminocytologic disassociation.
Guillain-Barré syndrome (GBS)
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
GBS Symptoms
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
GBS Diagnostics
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
GBS Management
Hospitalization to monitor progressive weakness, provide supportive care, and manage potential complications is necessary in all but the mildest suspected cases of GBS.
