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Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021
| Contributor Information
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Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Vertigo is the illusion of movement. The patient feels that the environment is moving or spinning.
Traditionally, vertigo is divided into two types: central, when the cause is a lesion in the central nervous system (CNS), and peripheral, when the cause is a lesion in the peripheral nervous system. However, in clinical practice, vertigo caused by lesions of cranial nerve VIII (acoustic nerve) is also considered central vertigo.[1-6]
The above magnetic resonance imaging (MRI) scan with gadolinium reveals a small acoustic neuroma within the left internal auditory canal.
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Central vertigo results from injury to the CNS structures related to the vestibular system in the cerebellum, the vestibular nuclei, or the connections within the brainstem. The causes of such injury include the following:
Vascular accidents, ischemic or hemorrhagic, mostly in the posterior circulation of the brain
CNS tumors
CNS infections
Trauma
Demyelinating disorders
Drug intoxication
The proton density–weighted magnetic resonance imaging (MRI) scan shown above reveals hyperintensity of the right temporal lobe in a patient with herpes encephalitis, which is a potential cause of vertigo.
Images courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
A 25-year-old woman presented for evaluation because of persistent episodes of vertigo over the preceding 7 days. About 7 months before the current presentation, she demonstrated weakness in the right leg, which improved but never returned to baseline strength. In addition to leg weakness, the patient also complained of fatigue, which was particularly intense after exercise or a hot shower.
Clinical examination showed mild weakness in the right leg, mild impairment on the finger-to-nose test, diplopia, and some dysarthria. MRI with fluid-attenuated inversion recovery (FLAIR) sequence revealed multiple T2-hyperintense white-matter lesions (left image) consistent with multiple sclerosis (MS). A follow-up MRI scan 3 months later (right image) showed a reduction in the size of these lesions, which is a common finding in patients with MS.
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
An axial proton density–weighted MRI through the posterior fossa in a patient with MS demonstrates multiple bright foci in the brainstem and cerebellum. Proton density–weighted sequences are highly sensitive for the detection of plaques in MS, especially in the posterior fossa.[3,7,8] MRI allows for better resolution than computed tomography (CT), without bony artifact, and is preferred over CT for imaging lesions in the posterior fossa.[9,10]
Which one of the following statements about MS is NOT true?
MS is an inflammatory demyelinating disease of the CNS.
MS is caused by a prion acquired in early life that remains dormant until it is activated by an acute infection.
MS is a dynamic disease with different clinical courses; even though some patients improve after the first attack, it should be considered a progressive neurologic disorder.
Central vertigo could be the presenting sign of MS in a small number of patients.
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Answer: B. No etiologic agent for MS has been identified, although low serum levels of vitamin D, smoking, childhood obesity, and infection with Epstein-Barr virus are likely to play a role in disease development.[11] Genetic susceptibility factors may play a role, in that the disease is more common in White populations living in northern latitudes. Spinal MS is often also present in patients with intracranial plaques.
A sagittal T2-weighted MRI scan of the cervical spine reveals increased signal in the cervical spine (arrow).
Image courtesy of Lars Grimm, MD.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
The MRI scan shows an ependymoma of the fourth ventricle (circle) compressing the cerebellum and brainstem.
In this patient, which one of the following symptom profiles is NOT likely to occur:
Headaches that occur in the morning, with some improvement during the day
Signs of cerebellar dysfunction, such as poor balance, gait disturbance, or ataxia
Epileptic seizures, most often focal, with secondary generalization
Vertigo, nausea, and other signs of brainstem dysfunction, such as diplopia, facial weakness, or tinnitus
Image courtesy of Lars Grimm, MD.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Answer: C. Epileptic seizures are not an expected finding in infratentorial lesions. However, they are more common with supratentorial tumors.
The above image, a sagittal slice from an MRI scan of a 9-year-old patient, shows an ependymoma arising from the fourth ventricle (circle), with resultant ventricular dilatation (arrow). Ependymomas are slow-growing tumors that develop from the ependymal cells and tend to invade locally. The majority of these tumors are infratentorial. When ependymomas occur in the posterior fossa, they invade the brainstem and adjacent structures, with some reaching the medulla and the upper spinal cord.[12] The high-grade tumors may metastasize through the cerebrospinal fluid.
The signs and symptoms of ependymomas can be subtle in the early stages and may be limited to headaches (primarily in the morning), behavioral changes, and vomiting (mostly related to increased intracranial pressure). Pressure on and direct infiltration of the brainstem can result in dysfunction of many cranial nerves.
Image courtesy of Lars Grimm, MD.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
The FLAIR MRI scan demonstrates a large brainstem glioma with associated mass effect.
The clinical symptoms and signs of brainstem gliomas are not different from those of ependymomas. In general, the clinical picture in brainstem glioma is related to the location of the tumor rather than to the tumor itself, with the triad of cranial nerve deficits, long-tract signs (eg, balance difficulties with wide-based gait, weakness, and hyperreflexia), and ataxia (of trunk and limbs) being strongly suggestive of brainstem pathology (most probably tumor). Central vertigo is a common sign of brainstem glioma, but it is not necessarily the initial sign. Brainstem gliomas are highly aggressive.
Image courtesy of Lars Grimm, MD.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
A 45-year-old man presented for evaluation because he had noticed a ringing sound in his right ear. For a while, his hearing on that side fluctuated, with periods of poor hearing followed by periods of spontaneous improvement. Occasionally, he experienced dizziness and headaches.
The axial MRI scan with gadolinium suggests what condition as the likely cause of his symptoms?
Image courtesy of Lars Grimm, MD.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Answer: The clinical picture and the MRI findings of an enhancing mass within the internal auditory canal point to an acoustic neuroma (vestibular schwannoma) (circle) as the likely cause.
Acoustic neuromas are intracranial extra-axial tumors that arise from the Schwann cells. When these tumors grow inside the internal auditory canal, they may compress cranial nerve VIII, as well as the labyrinthine artery, resulting in a combination of cochlear and vestibular symptoms.[3]
Hearing loss is the most common presenting symptom of an acoustic neuroma. Unilateral tinnitus, headache, ataxia, or vertigo may be present, but none of these is a common initial symptom in patients with acoustic neuromas.[13] Treatment is extremely challenging, given the location; however, individual centers have demonstrated some expertise with the use of stereotactic radiosurgery.[14]
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Neurofibromatosis type 2 (NF2) is an autosomal disorder resulting from a defective tumor suppressor gene on chromosome 22q12.2. It is a multisystem disease in which acoustic neuromas are common, and the presence of bilateral tumors (arrows) is a major clinical feature.[15] Cataracts, peripheral neurofibromas, and café-au-lait spots may also be present.
Images courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
NF2 is associated with different types of tumors. With the aim of differentiating NF2 more clearly from neurofibromatosis type 1 (NF1), some clinicians have proposed using the acronym MISME (multiple inherited schwannomas, meningioma, and ependymomas) syndrome for NF2.
The MRI scans are from two patients with NF2, neither of whom have acoustic neuromas. The tumors are identified with arrows. Which of these two images is more likely to be associated with central vertigo?
Images courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
The MRI scan on the left demonstrates a lesion (probably an ependymoma) in the brainstem, where it is most likely to affect the vestibular structures. The supratentorial lesions are likely to be meningiomas.
The MRI scan on the right demonstrates a lesion (most likely a meningioma) that is not in contact with the brainstem and thus is unlikely to cause central vertigo.
Image courtesy of Lars Grimm, MD.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Vertebrobasilar (posterior) circulation constitutes the arterial supply to the brainstem, cerebellum, and occipital cortex. Vertigo is the main symptom in patients with ischemia in the vertebrobasilar distribution.[16]
Given that a high concentration of cranial nerve nuclei, motor/sensory tracts, and the activating reticular system are situated in the brainstem, deficiencies in vertebral circulation result in complex and varied neurologic syndromes. Crossed signs (eg, contralateral motor and sensory findings) are hallmarks of many types of brainstem strokes. The most feared complication of brainstem lesions is sudden death, most likely due to acute cessation of respirations.
Central vertigo is a frequent manifestation of cerebellar hemorrhage. In the early stages of the disease, however, only subtle, transient signs and symptoms may be present. The CT scan shows acute cerebellar hemorrhage (circle).
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
A 65-year-old woman with a long history of type 2 diabetes mellitus, obesity, and hypertension arrived at the emergency department (ED) confused and with the acute onset of dizziness, gait disturbance, and headaches. In the ED, vomiting was observed, and the patient experienced a rapid deterioration in mental status. A noncontrast head CT scan revealed a large right cerebellar hemorrhage (shown).
Which one of the following statements is NOT true?
Emergency surgery is indicated in all cases of cerebellar hemorrhage.
Spontaneous cerebellar hemorrhage is an important, life-threatening cause of vertigo.
Ataxia would be an important clinical finding in this patient.
Long-standing hypertension and rupture of small penetrating vessels are the most probable cause in two thirds of cases.
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Answer: A. There is no uniform consensus about the most appropriate treatment of cerebellar hemorrhage except in extreme cases.[17] However, there is general agreement that if the patient is awake and has a Glasgow Coma Scale score of 14 or higher (9 for some), with a small bleed (for some, diameter <30 mm; for others, diameter <40 mm) and no hydrocephalus, conservative supportive care with close monitoring may be the best approach. Also, most clinicians agree that nonsurgical management and supportive care are indicated for a patient who is comatose, flaccid, and without brainstem reflexes.
An MRI scan shows an acute cerebellar hemorrhage in a different patient.
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
A 28-year-old man presented in the ED because of repeated episodes of acute vertigo that occurred after chiropractic neck manipulations for chronic neck pain. Results of the initial physical examination were normal, and the patient was discharged. However, the following day, in addition to vertigo, the patient had loss of balance, blurred vision, and mild numbness and weakness of the left extremities, with cerebellar signs on the right side. Angiography revealed a dissection of the vertebral artery (arrow).
Which one of the following statements is NOT true?
Vertebral artery dissection is a major cause of brainstem stroke in older adults.
Most vertebral artery dissections are associated with sudden neck movements.
Chiropractic neck manipulations could be a cause of vertebral artery dissection.
Images courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Answer: A. Vertebral artery dissection is not a major cause of brainstem ischemia in older adults. It is, however, an important cause of brainstem stroke in young patients. Chiropractic neck manipulations, as well as other types of minor trauma, have been described preceding both vertebral and carotid artery dissections, but a causal link has not been confirmed.[18]
Clinical symptoms consistent with vertebral artery dissection start in 72% of patients within 2 days of the traumatic event.[19] In some cases, however, it may take longer for the symptoms to develop. Dissection most commonly presents with neck pain, which may radiate to the head.
Most traumatic dissections involve the atlanto-occipital segment.[20] The prognosis is poorer when the dissection is the result of a traumatic condition and somewhat better in cases of spontaneous dissection.
This patient was treated with aspirin and clopidogrel, with complete resolution in 6 months (right image).
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
A diffusion-weighted MRI scan reveals restricted diffusion in the left medial temporal lobe (arrow), which is a classic pattern for herpes encephalitis.[21]
A 14-year-old boy presented with a 5-day history of fever and sore throat, followed by vertigo, nausea, and vomiting 3 days later. At admission, he had hyperemia in the oropharynx and vesicles on the upper lip. During the neurologic examination, lethargy, gait unsteadiness, nystagmus, and absence of the vomiting reflex were observed. Diagnostic studies demonstrated an infection with herpes simplex virus (HSV) type 1 (HSV-1). He was treated with acyclovir and showed improvement.
Which one of the following statements about herpes encephalitis is NOT true?
HSV encephalitis is a potentially fatal infection of the CNS.
Acyclovir reduces both mortality and morbidity in HSV encephalitis.
HSV is the most common cause of sporadic encephalitis in the United States.
In the pediatric population, HSV-1 encephalitis restricted to the brainstem is the most common form of encephalitis.
The gold standard for confirming HSV encephalitis is the detection of HSV DNA by polymerase chain reaction (PCR) assay.
Image courtesy of Medscape.
Central Vertigo: Identifying the Hidden Cause
Norberto Alvarez, MD | November 9, 2021 | Contributor Information
Answer: D. In the pediatric population, HSV-1 encephalitis restricted to the brainstem is NOT the most common form of encephalitis.
In children older than 3 months, as in adults, HSV-1 encephalitis occurs primarily in the temporal and frontal lobes, whereas neonates with herpes encephalitis typically acquire HSV-2 at delivery, with generalized brain involvement.[21] Early diagnosis of herpes encephalitis is crucial because the prognosis improves with early use of acyclovir. The gold standard for diagnosis is detection of DNA by means of PCR assay, although false-negatives have been reported.
The presence of vertigo in this case is related to the involvement of vestibular structures in the brainstem.
A T1-weighted MRI scan from a different patient reveals cortical hyperintensity from petechial hemorrhage (arrows), which is a less common finding in herpes encephalitis.
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Contributor Information
Author
Norberto Alvarez, MD
Assistant Professor, Department of Neurology
Harvard Medical School;
Consulting Staff, Department of Neurology
Boston Children's Hospital
Boston, MA
Disclosure: Norberto Alvarez, MD, has disclosed no relevant financial relationships.
Editor
Lars Grimm, MD
Associate Professor
Department of Diagnostic Radiology
Duke University Medical Center
Durham, NC
Disclosure: Lars Grimm, MD, has disclosed no relevant financial relationships.
Reviewer
Keith A. Marill, MD, MS
Associate Professor
Harvard Medical School;
Faculty, Department of Emergency Medicine
Massachusetts General Hospital
Boston, MA
Disclosure: Keith A. Marill, MD, MS, has disclosed the following financial relationships: Received ownership interest from General Electric and Medtronic.
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References
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