Amyotrophic lateral sclerosis physical examination
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]
Overview
Physical Examination
- ALS leads to progressive degeneration of the motor neurons that supply voluntary muscles, including Lower motor neurons in the medulla and anterior horn of the spinal cord as well as Lower motor neurons in the cerebral cortex.
- Physical examination of patients with amyotrophic lateral sclerosis is usually remarkable for:
- Lower motor neuron signs:
- Upper motor neuron signs:
- Spasticity
- Hyperreflexia
- Weakness
- Positive Babinski sign
- Positive Hoffmann sign
- Emotional lability
- ALS begins in the limbs, usually the arms, in about two-thirds of patients. The first symptoms are most often unilateral and focal.
- . As limb function deteriorates, patients become dependent on caregivers. They may fall and lose the ability to walk. Bulbar-onset ALS, which is more frequent in older women, carries a worse prognosis [23]. The first symptom is often dysarthria followed by dysphagia, which may progress to sialorrhea, malnutrition and anarthria. An atrophied fasciculating tongue is practically diagnostic of bulbar ALS. Axial weakness can cause dropped head and kyphosis, which are associated with pain and poor balance. Sphincter and sensory functions are usually, but not always, spared. Eye movements are preserved until advanced stages.
Cognitive impairment in ALS was described by Pierre Marie in the 19th century [24], but was considered uncommon until recently. Overt frontotemporal dementia (FTD) occurs in approximately 15% of people with ALS, but up to 50% are classified as impaired if measured by neuropsychological tests [25,26]. Primary progressive aphasia, semantic dementia and the behavioral variant are subtypes of FTD that affect executive function, language, judgment, personality, and behavior. Patients with ALS and dementia have shorter survival, possibly as a result of indecisiveness about care [27].
Depression and anxiety can occur during any stage of the disease, from time of diagnosis to respiratory failure, but patients with ALS often approach the disease philosophically and rates of depression seem to be lower than expected [28]. When present, emotional symptoms impair quality of life through poor sleep and appetite, as well as feelings of hopelessness [29].
Pain can occasionally result from involvement of sensory neurons, and frequently from contractures, immobility, inability to turn in bed, or bedsores. The suffering that arises from being unable to move can be intense (www.nybooks.com/articles/archives/2010/jan/14/night).
Morning headache, weak cough, orthopnea, and exertional dyspnea are early respiratory symptoms. As the disease advances, shortness of breath occurs during simple tasks such as dressing and eating, and eventually at rest.
The diagnosis, which depends on progressive UNM and LMN findings by history and examination, is accurate 95% of the time when made by an experienced clinician [30]. Electromyography confirms widespread LMN disease and excludes other diseases such as multifocal motor neuropathy with conduction block. Brain and spinal MRI rule out conditions that affect the UMN, including cervical spondylosis. Occasionally the brain MRI shows bilateral signal changes within the corticospinal tracts, a finding that is pathognomonic of ALS.
The El Escorialcriteria help standardize diagnosis for clinical research studies[31] (Table 1). Progressive LMN disease by clinical and electromyographic examination, and clinical UMN signs are the core. Patients are classified by the number of involved body regions: bulbar, cervical, thoracic or lumbosacral. Recent modifications created on Awaji Island near Japan may improve diagnostic sensitivity, particularly for those with bulbar-onset in whom limb findings can be subtle [32].
Vital Signs
- High-grade / low-grade fever
- Hypothermia / hyperthermia may be present
- Tachycardia with regular pulse or (ir)regularly irregular pulse
- Bradycardia with regular pulse or (ir)regularly irregular pulse
- Tachypnea / bradypnea
- Kussmal respirations may be present in _____ (advanced disease state)
- Weak/bounding pulse / pulsus alternans / paradoxical pulse / asymmetric pulse
- High/low blood pressure with normal pulse pressure / wide pulse pressure / narrow pulse pressure
Skin
- Skin examination of patients with [disease name] is usually normal.
OR
-
Description (Adapted from Dermatology Atlas)
-
Description (Adapted from Dermatology Atlas)
HEENT
- HEENT examination of patients with [disease name] is usually normal.
OR
- Abnormalities of the head/hair may include ___
- Evidence of trauma
- Icteric sclera
- Nystagmus
- Extra-ocular movements may be abnormal
- Pupils non-reactive to light / non-reactive to accommodation / non-reactive to neither light nor accommodation
- Ophthalmoscopic exam may be abnormal with findings of ___
- Hearing acuity may be reduced
- Weber test may be abnormal (Note: A positive Weber test is considered a normal finding / A negative Weber test is considered an abnormal finding. To avoid confusion, you may write "abnormal Weber test".)
- Rinne test may be positive (Note: A positive Rinne test is considered a normal finding / A negative Rinne test is considered an abnormal finding. To avoid confusion, you may write "abnormal Rinne test".)
- Exudate from the ear canal
- Tenderness upon palpation of the ear pinnae/tragus (anterior to ear canal)
- Inflamed nares / congested nares
- Purulent exudate from the nares
- Facial tenderness
- Erythematous throat with/without tonsillar swelling, exudates, and/or petechiae
Neck
- Neck examination of patients with [disease name] is usually normal.
OR
- Jugular venous distension
- Carotid bruits may be auscultated unilaterally/bilaterally using the bell/diaphragm of the otoscope
- Lymphadenopathy (describe location, size, tenderness, mobility, and symmetry)
- Thyromegaly / thyroid nodules
- Hepatojugular reflux
Lungs
- Pulmonary examination of patients with [disease name] is usually normal.
OR
- Asymmetric chest expansion OR decreased chest expansion
- Lungs are hyporesonant OR hyperresonant
- Fine/coarse crackles upon auscultation of the lung bases/apices unilaterally/bilaterally
- Rhonchi
- Vesicular breath sounds OR distant breath sounds
- Expiratory wheezing OR inspiratory wheezing with normal OR delayed expiratory phase
- Wheezing may be present
- Egophony present/absent
- Bronchophony present/absent
- Normal/reduced tactile fremitus
Heart
- Cardiovascular examination of patients with [disease name] is usually normal.
OR
- Chest tenderness upon palpation
- PMI within 2 cm of the sternum (PMI) / Displaced point of maximal impulse (PMI) suggestive of ____
- Heave / thrill
- Friction rub
- S1
- S2
- S3
- S4
- Gallops
- A high/low grade early/late systolic murmur / diastolic murmur best heard at the base/apex/(specific valve region) may be heard using the bell/diaphgram of the stethoscope
Abdomen
- Abdominal examination of patients with [disease name] is usually normal.
OR
- Abdominal distention
- Abdominal tenderness in the right/left upper/lower abdominal quadrant
- Rebound tenderness (positive Blumberg sign)
- A palpable abdominal mass in the right/left upper/lower abdominal quadrant
- Guarding may be present
- Hepatomegaly / splenomegaly / hepatosplenomegaly
- Additional findings, such as obturator test, psoas test, McBurney point test, Murphy test
Back
- Back examination of patients with [disease name] is usually normal.
OR
- Point tenderness over __ vertebrae (e.g. L3-L4)
- Sacral edema
- Costovertebral angle tenderness bilaterally/unilaterally
- Buffalo hump
Genitourinary
- Genitourinary examination of patients with [disease name] is usually normal.
OR
- A pelvic/adnexal mass may be palpated
- Inflamed mucosa
- Clear/(color), foul-smelling/odorless penile/vaginal discharge
Neuromuscular
- Neuromuscular examination of patients with [disease name] is usually normal.
OR
- Patient is usually oriented to persons, place, and time
- Altered mental status
- Glasgow coma scale is ___ / 15
- Clonus may be present
- Hyperreflexia / hyporeflexia / areflexia
- Positive (abnormal) Babinski / plantar reflex unilaterally/bilaterally
- Muscle rigidity
- Proximal/distal muscle weakness unilaterally/bilaterally
- ____ (finding) suggestive of cranial nerve ___ (roman numerical) deficit (e.g. Dilated pupils suggestive of CN III deficit)
- Unilateral/bilateral upper/lower extremity weakness
- Unilateral/bilateral sensory loss in the upper/lower extremity
- Positive straight leg raise test
- Abnormal gait (describe gait: e.g. ataxic (cerebellar) gait / steppage gait / waddling gait / choeiform gait / Parkinsonian gait / sensory gait)
- Positive/negative Trendelenburg sign
- Unilateral/bilateral tremor (describe tremor, e.g. at rest, pill-rolling)
- Normal finger-to-nose test / Dysmetria
- Absent/present dysdiadochokinesia (palm tapping test)
Extremities
Physical examination of extremities in patients with amyotrophic lateral sclerosis is usually remarkable for:
- Lower motor neuron signs:
- Upper motor neuron signs:
- Spasticity
- Hyperreflexia
- Weakness
- Positive Babinski sign
- Positive Hoffmann sign
- Emotional lability
- Foot drop
- Difficulty walking