Vestibular Test Results of a Patient with Cerebral Microangiopathy and Idiopathic Thrombocytopenia
Background Cerebral microangiopathy (CM) is a general term for small ischemic changes related to small blood vessels in the brain. Immune thrombocytopenia (ITP) is a rare acquired autoimmune disease that is characterized by low platelet count and increased risk of hemorrhage. We describe vestibular testing completed on a 64-year-old patient with CM and ITP presenting with complaints of general instability and listing while walking, decreased stability in dark environments, and blurred vision with head movement. Results indicated a severe loss of horizontal semicircular canal function bilaterally with a somatosensory-dependent pattern of postural instability.
Purpose This case emphasizes the importance of vestibular testing to determine the level of involvement of the vestibular system in the patient's symptomatology with known CM. Due to the increased risk of severe injury in those with ITP and the unknown effects this may have on the vestibular and balance systems, what is known about the pathophysiology of ITP and possible implications are reviewed.
Research Design Case report.
Data Collection and Analysis Previous audiological and medical records were obtained. Vestibular testing included videonystagmography; video head impulse test (vHIT); rotational chair including sinusoidal harmonic acceleration (SHA), step velocity, SHA with fixation, and visual vestibular SHA; computerized dynamic posturography battery including sensory organization test (SOT), motor control test, and adaptation test.
Results Audiological evaluation indicated a mild to moderately severe sensorineural hearing loss bilaterally. Ocular motor evaluation results indicated abnormal “cog-wheel” like tracings in smooth pursuit, hypometric saccades with abnormally increased latencies and reduced optokinetic gain. The patient also demonstrated apogeotropic nystagmus during head/body right and head/body left positions when fixation was denied. Bilateral bithermal calorics indicated a bilateral hypofunction. Horizontal vHIT results indicated reduced gain bilaterally. SHA results confirmed significant gain reductions at all frequencies tested and step velocity results indicated reduced gain and reduced time constants during all prerotary and postrotary measures. SOT results indicated a somatosensory-dependent pattern (i.e., reduced equilibrium index scores and falls in conditions 4, 5, and 6).
Conclusions These findings emphasize the importance of comprehensive vestibular evaluations of patients with CM, ITP, and complaints of instability. Identification and quantification of residual peripheral vestibular function as well as central system involvement can provide significant information pertinent to falls risk reduction and vestibular rehabilitation strategies, outcomes, and goals.
Keywordscerebral microangiopathy - computerized dynamic posturography - idiopathic thrombocytopenia - video head impulse test - videonystagmography - rotational chair - sinusoidal harmonic acceleration - bilateral hypofunction
Received: 24 July 2019
Accepted: 25 January 2020
27 April 2020 (online)
© 2020. American Academy of Audiology. This article is published by Thieme.
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- 1 de Leeuw FE, de Groot JC, Achten E. et al. Prevalence of cerebral white matter lesions in elderly people: a population based magnetic resonance imaging study. The Rotterdam Scan Study. J Neurol Neurosurg Psychiatry 2001; 70 (01) 9-14
- 2 Okroglic S, Widmann CN, Urbach H, Scheltens P, Heneka MT. Clinical symptoms and risk factors in cerebral microangiopathy patients. PLoS One 2013; 8 (02) e53455
- 3 Bath PM, Wardlaw JM. Pharmacological treatment and prevention of cerebral small vessel disease: a review of potential interventions. Int J Stroke 2015; 10 (04) 469-478
- 4 Feudjo-Tepie MA, Robinson NJ, Bennett D. Prevalence of diagnosed chronic immune thrombocytopenic purpura in the US: analysis of a large US claim database: a rebuttal. J Thromb Haemost 2008; 6 (04) 711-712 , author reply 713
- 5 Deng G, Yu S, Li Q. et al. Investigation of platelet apoptosis in adult patients with chronic immune thrombocytopenia. Hematology 2017; 22 (03) 155-161
- 6 Zimmer J, Andrès E, Noel E, Koumarianou A, Blicklé JF, Maloisel F. Current management of adult idiopathic thrombocytopenic purpura in practice: a cohort study of 201 patients from a single center. Clin Lab Haematol 2004; 26 (02) 137-142
- 7 Leigh R, Zee D. The Neurology of Eye Movements. 4th ed. New York, NY: Oxford University Press; 2006
- 8 Roberts RA. Technique and interpretation of positional testing. In: Jacobson GP, Shepard NT. eds. Balance Function Assessment and Management. 2nd ed. San Diego, CA: Plural Publishing; 2016
- 9 Macdonald NK, Kaski D, Saman Y, Al-Shaikh Sulaiman A, Anwer A, Bamiou D-E. Central positional nystagmus: a systematic literature review. Front Neurol 2017; 8 (April): 141
- 10 Lucieer F, Vonk P, Guinand N, Stokroos R, Kingma H, van de Berg R. Bilateral vestibular hypofunction: insights in etiologies, clinical subtypes, and diagnostics. Front Neurol 2016; 7: 26
- 11 Cerchiai N, Mancuso M, Navari E, Giannini N, Casani AP. Aging with cerebral small vessel disease and dizziness: the importance of undiagnosed peripheral vestibular disorders. Front Neurol 2017; 8: 241