OBJECTIVES: To highlight the clinical significance of de novo dystonia and its spontaneous resolution during pregnancy. METHODS: Two patients were evaluated in a movement disorder clinic during their first trimester of pregnancy and followed up longitudinally. RESULTS: Our first patient developed severe right torticollis and laterocollis during the sixth week of pregnancy. No etiology could be identified by a series of laboratory and imaging investigations for dystonia. The patient had near-complete resolution of cervical dystonia, without any therapeutic interventions. The second patient developed cervical dystonia and severe neck spasms during the eighth week of pregnancy, and no identifiable etiologies were found. Because of the extreme severity of dystonia, the patient had to undergo medical termination of pregnancy, after which the dystonia subsided. DISCUSSION: The literature on de novo dystonia in pregnancy or “dystonia gravidarum” is limited. To date, only three such cases have been reported. Our report expands the literature on this rare phenomenon. The cases in our study highlight that new movement disorders during pregnancy may indicate aberrant dopamine receptor sensitivity in a hyperestrogenic environment and that they are often self-limiting.
Keywords: Dystonia, dystonia gravidarum, movement disorders, pregnancy
| Introduction|| |
A plethora of physiological changes during pregnancy may trigger the onset of several disorders. Restless legs syndrome and chorea gravidarum are the most commonly reported movement disorders in pregnancy; however, the occurrence of dystonia (dystonia gravidarum) is rare.,, In this case report, we describe two cases of possible dystonia gravidarum and review the literature on this rare entity.
| Case Reports|| |
A 28-year-old primiparous woman presented with a 1-week history of pain and abnormal movements in the neck in her sixth week of pregnancy. In addition, she had hyperemesis gravidarum, for which she had taken doxylamine. She had no chronic illness, and there was no family history of any chronic neurological illnesses. She was prescribed benzodiazepines and anticholinergics for the symptomatic management of dystonia, but there was no clinically gratifying benefit with the medications. On examination, she had torticollis and mild laterocollis towards the right side and a prominent left sternocleidomastoid. In addition, she experienced frequent painful spasms in her neck and back (Video 1). Further workup for metabolic derangements including comprehensive metabolic panel, serum copper, and ceruloplasmin was within normal limits. Antinuclear antibody and antiphospholipid antibodies were negative. Magnetic resonance imaging of the brain was normal. Clinically, the patient was considered to have acute cervical dystonia, and supportive therapy with benzodiazepine was maintained. During subsequent follow-up in the third trimester, she had complete resolution of her symptoms, which was maintained following uneventful delivery (Video 2, post-delivery). A final diagnosis of dystonia gravidarum was considered based on precipitation of acute cervical dystonia during the first trimester, which spontaneously resolved by the third trimester.
A 30-year-old woman experienced turning of the neck towards the right side resulting in significant difficulty in walking, during her eighth week of pregnancy (primigravida). Her abnormal neck posturing was associated with severe neck pain. There was no associated remarkable medical illness, and her family had no history of any chronic neurological illness. Neurologically, she had severe right torticollis, with painful spasms. Complete blood count, comprehensive metabolic panel, serum copper and ceruloplasmin, antiphospholipid/antinuclear antibody profile, and magnetic resonance imaging of the brain were normal. The patient preferred to undergo medical termination of pregnancy (MTP) in the fourth month due to extreme cervical discomfort. Within 24 hours of MTP, her symptoms started to improve and within approximately 8–10 days, cervical dystonia completely resolved (video is unavailable). Clinical assessment 2 years following the event revealed only mild torticollis (<5 degrees) to the right.
| Discussion|| |
Dystonia gravidarum is a rare entity, with only three published reports available in the literature,, ([Table 1]). Lim et al. reported the first case in 2005, in which they described a 31-year-old primigravida, primiparous woman who developed acute cervical dystonia 4 weeks after amenorrhea. She continued to improve throughout the pregnancy, and by the third trimester, she reported complete resolution of dystonia. Given the spontaneous remission, the term “dystonia gravidarum” was proposed by the authors in relation to the well-known entity “chorea gravidarum.” Subsequently, two other studies reported de novo dystonia in two patients, which emerged during the first trimester of pregnancy and remarkably improved by the end of pregnancy., Identification of these cases has stimulated the debate on whether or not dystonia gravidarum is a unique entity. The cases in our report further enrich the literature on this rare condition. Similar to previously published reports, our patients experienced onset of cervical dystonia in the first trimester. Although it resolved by the third trimester for the first patient, medical termination of pregnancy resulted in improvement of dystonia for the second patient.
Although the precise etiology and pathogenesis of dystonia during pregnancy remain elusive, the role of a hyperestrogenic state has been speculated. This speculation stems from the fact that the onset of dystonia in all the previous reports was in the first few weeks of pregnancy, during which the estrogen levels are high; the improvement begins in the third trimester, which is a relatively hypoestrogenic state. This phenomenon is possibly related to the aberrant dopamine receptor sensitivity in the background of high estrogen levels. Similar postulations have been put forth to explain chorea gravidarum and worsening of pre-existing dystonia, such as hemimasticatory spasms during pregnancy.
Despite our promising findings, our study has several limitations. We acknowledge that although our cases can be described under the rubric “dystonia gravidarum,” it is possible that our patients may have had underlying neurological disorders, which surfaced during the pregnancy and resolved or became nonapparent again after the completion/termination of pregnancy. While we ruled out the common metabolic, structural, and immunological etiologies, genetic testing was not performed to identify genetic forms of dystonia, which is one of the limitations of this case series. We did not suspect other rare etiologies in the autoimmune or paraneoplastic spectrum, since the resolution of symptoms without any immunomodulating treatment is highly unlikely for such etiologies. Furthermore, we have the video demonstration for the first case but not for the second. This may be considered a limitation, especially because although dystonia improved to a large extent after MTP, it remained clinically observable.
In conclusion, dystonia gravidarum appears to be a rare distinct entity, which is perhaps underreported or underdiagnosed. This represents the onset of new dystonia or recurrence of dystonia or worsening of pre-existing dystonia that predominantly affects the cervical muscles. It usually manifests in the first trimester and may resolve in the third or immediately after delivery or termination of pregnancy. It is unclear why only a subset of patients develop dystonia during pregnancy. There may be a pre-existing susceptibility to dystonia, which gets a second hit in terms of a hyper-estrogenic state during pregnancy. Thorough longitudinal assessments of more such cases will provide additional insights into this enigmatic phenomenon.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
- (1)Research Project: A. Conception, B. Organization, C. Execution
- (2)Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique.
AL: 1B, 1C, 2A
LKP: 1A, 1B, 1C, 2B
Ethical compliance statement
Institute ethics review board approval was not recommended as this was retrospective report of two clinical cases.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Ba F, Miyasaki JM. Movement disorders in pregnancy. Handb Clin Neurol 2020;172:219-39.
Lim ECH, Seet RCS, Wilder-Smith E, Ong BKC. Dystonia gravidarum: A new entity? Mov Disord 2006;21:69-79.
Fasano A, Elia AE, Guidubaldi A, Tonali PA, Bentivoglio AR. Dystonia gravidarum: A new case with a long follow-up. Mov Disord 2007;22:564-6.
Buccoliero R, Palmeri S, Malandrini A, Dotti MT, Federico A. A case of dystonia with onset during pregnancy. J Neurol Sci 2007;260:265-6.
Hruska RE, Silbergeld EK. Increased dopamine receptor sensitivity after estrogen treatment using the rat rotation model. Science 1980;208:1466-8.
Cersósimo MG, Bertoti A, Roca CU, Micheli F. Botulinum toxin in a case of hemimasticatory spasm with severe worsening during pregnancy. Clin Neuropharmacol 2004;27:6-8.
Department of Neurology, Center for Parkinson’s Disease and Movement Disorders, Manipal Hospital, Millers Road, Bengaluru, Karnataka.
Source of Support: None, Conflict of Interest: None