Mal de débarquement syndrome (MdDS) is a rare cause of imbalance encountered in a neurology practice. However, it consists of classic features that the practicing neurologist should be able to recognize when evaluating a patient with dizziness. It is characterized by a feeling of rocking and swaying, and typically follows prolonged exposure to motion, such as being on a boat or plane. In this review article, we provide the clinical neurologist with the history of this disorder followed by illustrative cases of patients diagnosed with MdDS. Next we present proposed criteria for aiding in diagnosis. Finally, we will discuss a differential diagnosis for the syndrome, insights into the possible pathophysiology, current treatments, and future directions in management.
We describe three patients diagnosed with bilateral vestibular dysfunction associated with the jet propellant type-eight (JP-8) fuel exposure. Chronic exposure to aromatic and aliphatic hydrocarbons, which are the main constituents of JP-8 military aircraft jet fuel, occurred over 3–5 years’ duration while working on or near the flight line. Exposure to toxic hydrocarbons was substantiated by the presence of JP-8 metabolite n-hexane in the blood of one of the cases. The presenting symptoms were dizziness, headache, fatigue, and imbalance. Rotational chair testing confirmed bilateral vestibular dysfunction in all the three patients. Vestibular function improved over time once the exposure was removed. Bilateral vestibular dysfunction has been associated with hydrocarbon exposure in humans, but only recently has emphasis been placed specifically on the detrimental effects of JP-8 jet fuel and its numerous hydrocarbon constituents. Data are limited on the mechanism of JP-8-induced vestibular dysfunction or ototoxicity. Early recognition of JP-8 toxicity risk, cessation of exposure, and customized vestibular therapy offer the best chance for improved balance. Bilateral vestibular impairment is under-recognized in those chronically exposed to all forms of jet fuel.
PURPOSE OF REVIEW This article provides an overview of the numerous causes of vertigo and dizziness that are due to central nervous system (CNS) pathology and guides clinicians in formulating a differential diagnosis and treating patients with CNS causes of vertigo. RECENT FINDINGS Specific autoimmune vestibulocerebellar syndromes may now be tested for, and this article discusses the antibodies known to cause such syndromes. Superficial siderosis can be more accurately diagnosed with imaging studies, and treatment using iron chelation has recently been studied but has not yet been established as an effective treatment. Central autonomic network damage in the brain can cause central orthostatic hypotension in some neurodegenerative diseases, and medication has been approved for treatment. SUMMARY CNS causes of vertigo are numerous and important for clinicians to recognize. Examination findings are still an extremely valuable way to diagnose central vertigo; therefore, learning how to differentiate central from peripheral vertigo based on examination is an important skill. CNS causes of vertigo often have available treatments.
Mal de debarquement syndrome (MdDS) is a disorder of persistent vertigo characterized by a feeling of oscillation such as rocking, bobbing, or swaying. It is triggered by passive motion, typically by exposure to water, air, or land transportation. This syndrome affects middle-aged individuals who are predominantly women. MdDS presents as a balance disorder that carries significant risk of morbidity due to both the direct effects of balance impairment and associated symptoms of fatigue, cognitive slowing, and visual motion intolerance. The Barany Society will be publishing criteria for diagnosing persistent MdDS. In addition, more insight has been gained into the pathophysiology of MdDS, with current hypotheses pointing to a cerebral and cerebellar basis. Treatments have expanded beyond medication trials, and now include the use of noninvasive brain stimulation and readaptation of the vestibulo-ocular reflex.
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