Ataxia and Vertigo Prepared by Jason Pillet Presented by Dr. Donze
Introduction Ataxia is the failure to produce smooth intentional movements. Gait disorders include ataxic gait as well as a variety of other conditions. The presenting complaint may be weakness, dizziness, stroke, falling, or another nonspecific complaint.
Pathophysiology Ataxia may categorized into motor and sensory ataxias. Motor ataxias (cerebellar ataxias) are usually caused by disorders of the cerebellum. The sensory receptors and afferent pathways are intact, but integration of the proprioceptive information is faulty. Involvement of the lateral cerebellum may lead to a motor ataxia of the ipsilateral limb. Lesions affecting the midline portion of the cerebellum cause problems with axial muscle coordination reflected in difficulty maintaining a steady upright standing or sitting posture.
Pathophysiology Sensory ataxias occur with failure in transmission of proprioception or position sense information to the CNS. This may arise from disorders affecting the peripheral nerves, spinal cord, or cerebellar input tracts. Sensory ataxias may be compensated to a degree with visual sensory information. Loss of visual information leads to the observation that sensory ataxia often worsen in poor lighting conditions and may by brought out during examination.
Clinical Features Obtain orthostatic vital signs Gait testing - Observe the patient sit upright in the stretcher, rise, stand, walk, and turn around. The patient should be asked to walk at a normal speed, then walk on the heels, and then toes. Tandem gait is toe-to-toe walking and tests many elements of the nervous system. Cerebellar functions are tested by asking the patient to perform smooth voluntary movements and rapidly alternating movements: dyssynergia (breakdown of movements into parts), dysmetria (inaccurate fine movements), or dysdiadochokinesia (clumsy rapid movements) may be indicative of a problem in the lateral cerebellum.
Clinical Features A test for cerebellar function that emphasizes the lower extremities is the heel-to-shin test. Romberg test is primarily a test of sensation and, if positive, may distinguish sensory from motor ataxia. The inability to maintain a steady standing posture confirms that an ataxia is present but does not give information about the type of ataxia. If the ataxia worsens with eyes closed, then the Romberg sign is present, suggesting sensory ataxia with a problem of proprioceptive input (posterior column, vestibular dysfunction), or a peripheral neuropathy. In patients who show no change in their unsteadiness with eye closure (Romberg negative), a motor ataxia is suggested with possible localization of that problem to the cerebellum
Tabes Dorsalis (Neurosyphilis) Sensory ataxia. loss of proprioceptive information from the lower extremities renders the patient dependent on visual cues for correct gait. The classic description is of a patient who walks slowly with wide gait while staring at the ground. In darkness or with interruption of vision, the patient is unable to walk. The gait is peculiar with the foot first raised and then slapped to the ground with each step. These abnormalities reflect the loss of proprioceptive information from the posterior roots and posterior columns.
Clinical Features A cerebellar or motor ataxic gait is wide based with unsteady and irregular steps, and compensation to barriers in the environment may be lacking. The gait of sensory ataxia resulting from loss of proprioception is notable for abrupt movement of the legs and slapping impact of the feet with each step.
Classification of Gait Disorders Low-level gait disturbance refers to disorders of proprioception or dysfunction of the musculoskeletal system. Middle-level gait disturbance causes distortion of appropriate interaction of postural and motor processes or synergies. This might include stroke with paralysis, cerebellar dysfunction, or diseases of the basal ganglia such as Parkinsons disease. On examination, patients might have findings of spasticity, muscular tone, paralysis, or abnormal movements. High-level gait disturbances involve structures or processes that choose the appropriate responses for the support surface, ie: Cautious gait, apraxic gait, and the frontal gait disorder conceptually fall into this group with pathology that correlates with lesions in the frontal cortex or thalamus.
Children and gait disturbances
Vertigo and dizziness Dizziness may mean vertigo, syncope, presyncope, weakness, giddiness, anxiety, or a disturbance in mentation to various patients. Vertigo is the perception of movement where no movement exists. Disequilibrium refers to a feeling of unsteadiness, imbalance, or a sensation of "floating" while walking.
Pathophysiology The central nervous system (CNS) coordinates and integrates sensory input from the visual, vestibular, and proprioceptive systems. The three streams of information help form an impression of the orientation of the head and body as well as the perception of motion. Vertigo arises from a mismatch of information from two or more of the involved senses, which, in turn, can be caused by dysfunction in the sensory organ or its corresponding pathway.
Pathophysiology Visual inputs provide spatial orientation. Proprioceptors help relate body movements and indicate the position of the head relative to that of the body. The vestibular system establishes the bodys orientation with respect to gravity. There are three semicircular canals which sense orientation to movement and head tilt and are filled with a fluid called endolymph. The movement of fluid causes specialized hair cells inside the canals to move, causing afferent vestibular impulses to fire which travels to the nucleus of the eighth cranial nerve.
Etiologies of Vertigo
Clinical Features Vertigo is usually categorized as "peripheral" or "central." Peripheral vertigo is caused by disorders affecting the vestibular apparatus and the eighth cranial nerve, whereas Central vertigo is caused by disorders affecting central structures, such as the brainstem and the cerebellum.
Peripheral vs CentralPeripheral vertigo tends to cause distressing symptoms, but is seldom life-threatening. Disorders causing central vertigo may produce lessdistressing symptoms and have a slower onset than those due to peripheral vertigo, but they are generally of a more serious nature
Diagnosis Peripheral vertigo is more likely than central vertigo to be intense and to be associated with nausea, vomiting, diaphoresis, tinnitus, hearing loss, and photophobia. Central vertigo is more likely to be associated with neurologic symptoms and signs such as diplopia, dysarthria, and bilateral visual abnormalities. An associated headache or history of headache suggests migraine, stroke, TIA or a space- occupying lesion.
Physical Exam Patients with vertigo should have ear, neurologic, and vestibular examinations. Insufflation of air by use of a pneumatic otoscope that precipitates a burst of vertigo with nystagmus is diagnostic of an inner ear fistula If central vertigo is considered, check for an absent corneal reflex, facial paresis, difficulty swallowing, dysphonia, and depressed gag reflex. Test for limb and truncal ataxia, and test the vestibulospinal system and cerebellum through tandem gait and Romberg testing.
Physical Exam Nystagmus is the principal objective sign of vertigo. The eyes should be examined for spontaneous nystagmus, and the direction of the fast component of nystagmus should be noted. The diagnosis of BPPV involving the posterior canal is aided by the Dix-Hallpike position test. http://www.accessmedicine.com.proxy.library.oh (how to perform the Dix-Hallpike)
Dix Hallpike Should not be performed on patients with carotid bruits, cerebrovascular disease, risk factors or concern for vertebrobasilar insufficiency, spinal injury, or cervical spondylosis. May provoke vertigo and pretreatment with 50 mg benadryl makes the test more tolerable but will not obliterate nystagmus. To test the right posterior semicircular canal, the head is initially rotated 30 to 45 degrees to the right. Keeping the head in this position, the patient is rapidly brought to the recumbent position until the head is 20 degrees below the level of the stretcher or examining table. A positive test is indicated by rotatory nystagmus following a latency of no more than 30 seconds; the nystagmus exhibits rapid eye torsions toward the affected ear and lasts for 10 to 40 seconds. The side exhibiting the positive test is the side of the lesion. The test is about 50% to 80% sensitive for BPPV
Treatment for Vertigo
Disorders causing Peripheral Vertigo Peripheral vertigo is noted for its abrupt (often explosive) onset. It is an intense sensation of spinning or hurtling toward the ground or surrounding walls. It is typically worsened by rapid movement and by changes in head position. It is frequently associated with nausea, often severe vomiting, diaphoresis, and bradycardia and hypotension.
BPPV Disorder of the inner ear causing transient vertigo and associated nystagmus that is precipitated by certain head movements According to the canalolithiasis hypothesis, BPPV is caused by inappropriate activation of a semicircular canal, typically the posterior semicircular canal and typically unilateral, by the presence of free-floating particles or otoconia. Average age of onset: mid-50s. Women are twice as likely to be affected. The onset is sudden, and an attack typically is precipitated by rolling over in bed, assuming a supine position, leaning forward, looking up at the sky or ceiling, or turning the head. Nausea is often present.
BPPV Because the symptoms fatigue, they tend to be worse in the morning and become less pronounced as the day progresses. Patients may eliminate the offending activities. There is no associated hearing loss or tinnitus, and no physical findings on examination of the external auditory canal.
BPPV treatment Medications such as transdermal scopolamine and antihistamines. Epley maneuver (particle-repositioning maneuver) principle behind the particle-repositioning maneuver is to use gravity to induce the particles to move along the semicircular canals until they end up inside the utricle, where they are unlikely to cause vertigo (http://www.accessmedicine.com.proxy.library.ohiou.edu/video Player.aspx?file=vid_epleymaneuver)
Epley Maneuver The patient is seated as in the Dix-Hallpike position test, and the head is turned 45 degrees toward the affected ear.
Epley Maneuver continued The patient is gently brought to the recumbent position with the head hanging roughly 20 degrees below the examining table. The head is gently rotated 45 degrees to the midline.
Epley Maneuver continued The head is then rotated a further 45 degrees to the unaffected side.
Epley Maneuver continued The patient rolls onto the shoulder of the unaffected side, at the same time rotating the head a further 45 degrees.
Epley Maneuver finished The patient is returned to the sitting position, and the head is returned to the midline. Each portion of the maneuver should be done slowly (about 5 minutes) and evenly to permit the particles to traverse their intended course. If the maneuver is done correctly, nystagmus in the same direction as that observed during Dix- Hallpike position testing may be observed.
Meniere Disease Associated with an increased endolymph within the cochlea and labyrinth Equally seen in men and women aged 65 and older and usually unilateral Onset of vertigo is usually sudden, with associated nausea, vomiting, and diaphoresis. The duration of vertigo ranges from 20 minutes to 12 hours Roaring tinnitus, diminished hearing, and fullness in one ear. Between attacks, the patient is usually well, although decreased hearing may persist
Meniere disease continued Diagnosis confirmed by gylyeral testing and by vestibular-evoked myogenic potentials. managed symptomatically with antihistamines, betahistine (H1 receptor agonist), and CCBs A salt-restricted diet <1 gram/d of added salt is recommended for patients with a confirmed diagnosis. Intratympanic gentamycin administration has been shown to provide significant immediate and long-term relief
Perilymph Fistula opening in the round or oval window that permits pneumatic changes in the middle ear to be transmitted to the vestibular apparatus Trauma, infection, or a sudden change in the pressure inside the ventricular system Diagnosis suggested by sudden onset of vertigo associated with flying, scuba diving, severe straining, heavy lifting, coughing, or sneezing Confirmed by nystagmus elicited by pneumatic otoscopy (Hennebert sign) Managed with symptomatic treatment and bed rest, with referral to an ENT
Vestibular Neuronitis Suspected viral etiology. Lasts several days and does not recur. Sudden onset The vertigo is often so intense that the patient requires several days of bed rest; Elderly patients may have persistent unsteadiness of gait. Unilateral loss of hearing and tinnitus may occur. Treated symptomatically.
Vestibular Ganglionitis Caused by varicella zoster that can be reactivated Multiple ganglia may be involved. Herpes zoster oticus, also known as the Ramsay Hunt syndrome, is a neuropathic disorder thought to be associated with vestibular ganglionitis. Characterized by deafness, vertigo, and facial nerve palsy. diagnosis is confirmed by the presence of grouped vesicles on an erythematous base inside the external auditory canal. Managed with a combination of symptomatic treatment and antiviral therapy started within 72 hours of the appearance of vesicles
Labyrinthitis Infection of the labyrinth producing peripheral vertigo and hearing loss. viral (measles and mumps), in which case the clinical course is similar to that of vestibular neuronitis. Bacteria (otitis media) in which bacteria and toxins diffuse across the membrane of the round window. A cholesteatoma can erode into the inner ear, creating a portal of entry for bacteria. The hallmarks of this disease include sudden onset of vertigo with associated hearing loss and middle ear findings. Patients with bacterial labyrinthitis are at risk for meningitis and require antibiotics and referral to an otologist or ENT specialist for admission and drainage.
Ototoxicity Ototoxicity has been associated with aminoglycoside and macrolide antibiotics, loop diuretics, platinum-based chemotherapeutic agents, some NSAIDs, and antimalarial preparations Aminoglycoside antibiotics produce hearing loss and peripheral vestibular dysfunction by accumulating inside the endolymph, where they cause the death of cochlear and vestibular hair cells. However, because both inner ears are affected, vertigo is uncommon
Eighth Nerve lesion may produce mild vertigo. Meningiomas and acoustic schwannomas are typical causes. The onset of vertigo is usually gradual, remaining constant until central compensation can take place. The vertigo is usually preceded by hearing loss. Such patients require urgent diagnostic imaging as well as referral to a neurosurgeon
Cerebellopontine Angle Tumors Associated with tumors of the cerebellopontine angle. include acoustic neuromas, meningiomas, and dermoids. They usually present with a cluster of findings, including deafness and ataxia, as well as ipsilateral facial weakness, loss of the corneal reflex, and cerebellar signs. Require urgent diagnostic imaging as well as referral to a neurosurgeon
Post-Traumatic Vertigo Acute post-traumatic vertigo is caused by a direct injury to the labyrinthine membranes. Onset is immediate and accompanied by nausea and vomiting. May have sustained a concomitant fracture of the temporal bone. Vertigo associated with a closed head injury warrants a CT scan or MRI to exclude an extradural or intradural hematoma. Vertigo due to direct labyrinthine trauma tends to resolve within several weeks. Postconcussive syndrome can be associated with unsteadiness of gait and a vague sense of dizziness.
Central Vertigo Disorders affecting the cerebellum and the brainstem. Gradual in onset and mild in intensity; symptoms are not provoked by changes in position. Unlikely to be associated with tinnitus and hearing impairment. Nystagmus is more likely to be vertical
Cerebellar Hemorrhage & infarction Causes acute vertigo and ataxia. Headache, nausea, and vomiting may or may not be present. Sense of side-to-side or front-to-back motion. Truncal ataxia and may not be able to sit without support. Romberg testing and tandem gait will be abnormal. Occasionally, there may be a sixth cranial nerve palsy or conjugate eye deviation away from the side with the hemorrhage. Cerebellar infarction has a similar clinical presentation. Such patients require urgent diagnostic imaging, and those with cerebellar hemorrhage require emergent neurosurgical consultation.
Wallenberg Syndrome A lateral medullary infarction (Wallenberg syndrome) of the brainstem can cause vertigo. Ipsilateral findings include facial numbness, loss of corneal reflex, Horner syndrome, and paralysis or paresis of the soft palate, pharynx, and larynx (causing dysphagia and dysphonia). Contralateral findings include loss of pain and temperature sensation in the trunk and limbs. Occasionally, lesions of the sixth, seventh, and eighth cranial nerves can occur, causing vertigo, nausea, vomiting, and nystagmus. usually require urgent diagnostic imaging and referral to a neurologist
Vertebrobasilar insufficiency TIAs of the brainstem. Orthostasis can worsen symptoms. Vertigo may be of sudden onset and typically lasts from minutes to hours. VBI-induced vertigo can present by itself or be accompanied by diplopia, dysphagia, dysarthria, and bilateral loss of vision. Unlike other causes of central vertigo, VBI may be provoked by position. Turning the head partially occludes the ipsilateral vertebral artery. If the contralateral artery is stenotic, head turning could cause transient ischemia to the brainstem, resulting in VBI. A sufficient loss of brainstem circulation caused by a head turn could affect the reticular activating system, causing near-syncope or syncope. Patients with VBI should have imaging as well as referral to a neurologist
Vertebral Artery dissection Lead to a stroke involving the posterior circulation. Symptoms and signs of vertebral artery dissection include headache and vertigo, and a unilateral Horner syndrome may be present. Sudden (and often violent) rotation or extension of the neck may precipitate a dissection. Injuries may occur following high-velocity motor vehicle crashes, diving injuries, coughing, sneezing, and chiropractic neck adjustments. Emergency diagnostic imaging
Multiple Sclerosis Demyelinating disease can present with vertigo that tends to last several hours to several days or weeks and is usually nonrecurrent. Vertigo not usually intense, and nystagmus is often more prominent than the vertigo Ataxia or optic neuritis may be present or may have occurred previously. Diagnosis usually is confirmed using MRI. Patients with vertigo due to demyelination require urgent referral to a neurologist.
Migraine related Vertigo Managed symptomatically with antivertigo therapy but require neurologic referral. Ergotamine or sumatriptin should not be used in basilar migraine. Migraine prophylactic agents, such as B-blockers and CCBs, may be instituted in consultationwith a neurologist
Disposition Patients with peripheral vertigo may be discharged from the ED. All patients with a first episode of peripheral vertigo should be referred to their PCP or an otolaryngologist. Patients with BPPV who have had a particle- repositioning maneuver should be referred to an otologist or ENT Central causes of vertigo almost always require urgent diagnostic imaging and neurologic or neurosurgical consultation while in the ED and admission.
Osteopathic Medicine Modified Muncie technique is type of myofascial release administered inside the patients mouth Epley Maneuver Cervical Muscle energy or HVLA Cranial release