Clinical Neurology and Neurosurgery 158 (2017) 126

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Clinical Neurology and Neurosurgery
journal homepage: www.elsevier.com/locate/clineuro

Letter to the Editor
Chorea gravidarum associated with Moyamoya angiopathy treated with alpha-methyldopa

MARK

Dear Ladys and Sirs,
We recently published a first systematic study concerning movement symptoms in Moyamoya disease in this journal [1]. Moyamoya disease is a
cerebrovascular disorder characterized by bilateral progressive narrowing and occlusion of the distal internal carotid artery and adjacent basal
cranial vessels with development of characteristic collateral circulation. We recently published that movement symptoms are more often than
traditionally thought to be [1]. Several underlying mechanisms have been discussed, comprising cerebral ischemia and diffuse hypoperfusion as well
as hormonal factors [1]. Hyperventilation for example by singing can precipitate symptoms through vasoconstriction [2]. Increased sex hormones
during pregnancy may trigger chorea gravidarum by enhancing the dopaminergic sensitivity of the basal ganglia [3,4]. Choreatic movements in
Moyamoya angiopathy have been reported to be responsive to haloperidol, another therapeutic option is bypass surgery [1].
We want to add a case description of chorea gravidarum in a Moyamoya patient which subsided after initiating alpha-methyldopa. The patient of
Mhallami ethnicity was diagnosed with Moyamoya angiopathy in January 2012 at the age of 17. She already had a three-year history of arterial
hypertension. Repeated transient losses of consciousness after an increase of her antihypertensive medication in 2011 finally led to the diagnosis of
her cerebrovascular disorder. Further symptoms since then have been transient hypesthesias of her hands as well as limb shaking TIAs. Some of her
diagnostic findings point to an autoimmune disease mechanism in terms of Moyamoya syndrome as opposed to idiopathic Moyamoya disease. For
instance, she was diagnosed with an uveitis, has a renal artery stenosis and positive oligoclonal banding in her cerebrospinal fluid. She underwent
STA-MCA-bypass surgery combined with encephalosynangiosis on both sides in 2012. During her first pregnancy in 2012 she was already treated
with alpha-methyldopa for her arterial hypertension.
In March 2014, the 19-year-old-patient presented with choreatic movements of her left side, mainly affecting the upper limb, which already
lasted for three days. Two days earlier she noted a transient numbness of her left hand. She was pregnant in the 16th week of gestation. Considering a
hemodynamic mechanism in spite of intact bypasses and cerebral MRI showing no diffusion restriction, the patient was at first treated with infusions
and reduction of her antihypertensive medication, keeping her blood pressure slightly increased. Meanwhile the movement disorder continued
unimproved. We changed antihypertensive treatment from metoprolol to alpha-methydopa due to our pathophysiological assumption of positive
effects on chorea. After the first two doses of alpha-methyldopa the movement disorder subsided, in the next two days it completely suspended.
Although alpha-methyldopa is not established as treatment option for hyperkinetic movement disorders and even has been reported as a trigger
for generalized choreatiform movements in a patient with chronic renal failure [5], we presume that it might have alleviated the hemichorea through
disturbance of dopamin production.
Thus, at least in patients with arterial hypertension, alpha-methyldopa may be an effective treatment of chorea gravidarum, which is ascribed to
enhanced dopaminergic sensitivity of the basal ganglia.
References
[1] M. Kraemer, et al., Movement symptoms in European Moyamoya angiopathy—first systematic questionnaire study, Clin. Neurol. Neurosurg. 152 (2017) 52–56.
[2] S.H. Han, et al., Moyamoya disease presenting with singing induced chorea, J. Neurol. Neurosurg. Psychiatry 69 (6) (2000) 833–834.
[3] S.M. Kranick, et al., Movement disorders and pregnancy: a review of the literature, Mov. Disord. 25 (6) (2010) 665–671.
[4] A. Kim, et al., Consecutive pregnancy with chorea gravidarum associated with moyamoya disease, J. Perinatol. 29 (4) (2009) 317–319.
[5] E.M. Neil, A.K. Waters, Generalized choreiform movements as a complication of methyldopa therapy in chronic renal failure, Postgrad. Med. J. 57 (673) (1981) 732–733.
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Jens Platzen, Peter Berlit, Markus Kraemer
Department of Neurology, Alfried-Krupp-Hospital, Essen, Germany
E-mail address: markus.kraemer@krupp-krankenhaus.de

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Corresponding author at: Department of Neurology, Alfried Krupp von Bohlen und Halbach Hospital, Alfried-Krupp-Str. 21, 45117 Essen, Germany.

http://dx.doi.org/10.1016/j.clineuro.2017.04.023
Received 28 February 2017
Available online 06 May 2017
0303-8467/ © 2017 Elsevier B.V. All rights reserved.