Cervical dystonia due to cerebellar stroke

Abstract

Sir, Although poststroke dystonia is usually associated with lesions of the lentiform nucleus, 1 recent reports suggest that cerebellum plays a key role in the pathogenesis of dystonia.2,3 We describe a patient who developed cervical dystonia after ipsilateral cerebellar infarction. CASE REPORTA 48-year-old woman patient presented with a sudden onset of vertigo, vomiting, and ataxia. On admission, neurological examination revealed horizontal, bidirectional nystagmus and ataxia of the left limbs. Her previous medical history was unremarkable. She smoked 20 cigarettes per day.Emergency MRI findings were consistent with acute cerebellar infarction in the irrigation area of the left superior cerebellar artery (Fig. 1a,b). Standard biochemistry, erythrocyte sedimentation rate, and complete blood count were within the normal limits. Immunologic tests (rheumatoid factor, antineutrophilic cytoplasmic antibodies, anticardiolypin antibodies, antinuclear antibodies) were negative. Chest X-ray and electrocardiogram were normal. Heart ultrasound was normal and did not show signs of patent foramen ovale. Fundus was normal.On the second day of hospital stay, she started developing abnormal posturing of her head along with sustained involuntary contractions of the cervical muscles, and so the head was rotated to the right and down. She was discharged to stationary rehabilitation with almost complete recovery of left limb ataxia. Therapy with aspirin (100 mg) was introduced, and because of registered mild hypertension, ramipril (2.5 mg) in the morning was prescribed. However, involuntary movements progressed severely over the next few weeks, and so she could just temporarily move her head to the normal position, and hypertophy of the left sternocleiodomastoid muscle developed. Neck MRI was normal. Clonazepam (0.5 mg; three times daily) and baclofen (5 mg; three times daily) were introduced. This therapy led to slight improvement of symptoms. One month later, after the application of botulinum toxin, her symptoms improved significantly.

A Functional Magnetic Resonance Imaging Study of Head Movements in Cervical Dystonia

“…Clinical imaging studies using computed tomography and structural MRI have linked CD with focal lesions of cerebellar circuits (14, 47, 50, 51). A diffusion tensor imaging (DTI) study in individuals with craniocervical dystonia (dystonia of facial and neck muscles combined) indicated abnormal cerebellar microstructure and fiber organization, especially in the anterior cerebellum and vermis (52).…”

Dystonia and cerebellar degeneration in the leaner mouse mutant

“…For example, what types of abnormalities in the cerebellum may cause dystonia? Cerebellar lesions, such as those caused by stroke, most often result in ataxia (Caplan 2005; Deluca, Moretto et al 2011), though sometimes they are associated with dystonia (Rumbach, Barth et al 1995; Alarcon, Tolosa et al 2001; O'Rourke, O'Riordan et al 2006; Zadro, Brinar et al 2008; Waln and LeDoux 2010; Usmani, Bedi et al 2011). The majority of cerebellar degenerative syndromes are also associated with ataxia.…”

Evidence for Altered Basal Ganglia-Brainstem Connections in Cervical Dystonia

“…In addition to the basal ganglia, the pontine brainstem [1], [2], [3], [4], and cerebellum [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20] have been implicated in dystonia by numerous studies. As a synthesis of this literature and a number of other observations in dystonia, we recently hypothesized that the pallidal output neurons exhibiting extensive collateralization to the brainstem [21], [22], [23] are the neurons gating the functional system that is affected in dystonia.…”