Delayed cervicobrachial segmental dystonia secondary to ipsilateral cerebellar infarction

Vikram V Holla; Sudhakar Pushpa Chaithra; Shweta Prasad; Pramod Kumar Pal

doi:10.4103/AOMD.AOMD_32_20

CASE REPORTS

Year : 2021 | Volume : 4 | Issue : 2 | Page : 89-91

Delayed cervicobrachial segmental dystonia secondary to ipsilateral cerebellar infarction

Vikram V Holla¹, Sudhakar Pushpa Chaithra¹, Shweta Prasad², Pramod Kumar Pal¹
¹ Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India
² Department of Clinical Neurosciences and Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India

Date of Submission	22-Jun-2020
Date of Decision	26-Jul-2020
Date of Acceptance	01-Sep-2020
Date of Web Publication	31-May-2021

Correspondence Address:
Dr. Pramod Kumar Pal
Department of Neurology, National Institute of Mental Health & Neurosciences, Bengaluru 560029, Karnataka.
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AOMD.AOMD_32_20

Abstract

Dystonia is the most common delayed movement disorder poststroke with basal ganglia involvement seen in the majority of them. Dystonia usually develops within 1 year of stroke. We report a case of poststroke cervicobrachial segmental dystonia which developed after 9 years of ipsilateral cerebellar stroke. Dystonia can be a sequela of cerebellar stroke, and may occur even after a prolonged latency. This should be considered in a case of segmental dystonia with the past history of cerebellar stroke.

Keywords: Cerebellum, posterior circulation stroke, segmental dystonia, upper limb dystonia

How to cite this article:
Holla VV, Chaithra SP, Prasad S, Pal PK. Delayed cervicobrachial segmental dystonia secondary to ipsilateral cerebellar infarction. Ann Mov Disord 2021;4:89-91

How to cite this URL:
Holla VV, Chaithra SP, Prasad S, Pal PK. Delayed cervicobrachial segmental dystonia secondary to ipsilateral cerebellar infarction. Ann Mov Disord [serial online] 2021 [cited 2023 May 28];4:89-91. Available from: https://www.aomd.in/text.asp?2021/4/2/89/317378

Introduction

Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Secondary dystonia following a basal ganglia stroke has been commonly reported.^[1],[2] Dystonia can develop either during acute stroke or as a delayed complication month to rarely years after the stroke. Apart from the basal ganglia, lesions in the thalamus, brainstem, cerebellum, and spinal cord have also been implicated in secondary dystonia. Cerebellar lesions may occasionally cause dystonia and very few cases have been reported in the literature.^[1],[2] This report describes a case of right upper limb (UL) and cervical segmental dystonia that developed almost 9 years after a right-sided posterior circulation stroke.

Case History

A 49-year-old man presented with a 3-year history of abnormal posturing of the right UL. His symptoms initially started with difficulty writing, owing to which he was treated as writer’s cramp. Subsequently, the abnormal posturing involved other activities and hindered his daily routine. No posturing was noted at absolute rest, that is, while lying down or standing with the arm at rest. There were no abnormal movements in any other limbs, trunk, or face. The patient had sustained a stroke 9 years before the onset of the aforedescribed posturing. The stroke was characterized by sudden onset vertigo, vomiting, with right facial weakness, bulbar weakness, right hemiataxia, and left hemisensory impairment. On examination, he was diagnosed to have a right vertebral artery dissection with right posterior inferior cerebellar artery territory infarct involving the right lateral pontomedullary junction and cerebellar region. Following 2 years of treatment and rehabilitation, the patient had improved with residual right facial weakness and mild nondisabling left hemisensory impairment. There was no significant family history suggestive of dystonia.

On examination higher mental functions, cranial nerves and speech were normal except for the right lower motor neuron type of facial weakness. On motor system examination, the patient had normal tone power, deep tendon reflexes with a normal bilateral plantar response. Sensory system examination revealed reduced pain and temperature in the left half of body without involvement of the face suggesting right spinothalamic tract impairment. Cerebellar system examination was unremarkable except for a mild impairment in tandem gait. When both UL were at absolute rest, that is, hanging by the side of his body, there was no dystonia. When the arms were kept on the lap, there was the intermittent slow elevation of the right shoulder with the lateral shifting of the head toward the right. This was associated with the adduction of the arm with slow pronation at the elbow, which was more prominent when the arms were outstretched, and during any activity such as while writing [Video 1]. No dystonia was observed while walking, and he had a normal arm swing. There was no dystonia elsewhere in the body and there was no evidence of other movement disorder phenomenology. Examination of other systems was normal.

Work up for recent-onset dystonia revealed normal routine blood investigations, and copper studies were normal. MRI brain with MRA showed atrophy with gliosis and encephalomalacia of the right lateral pontomedullary junction, middle cerebellar peduncle, and right cerebellar hemisphere mainly posterior lobar region predominant bilateral cerebellar atrophy with occlusion of V4 segment of the right vertebral artery [Figure 1]. Final diagnosis of probable poststroke dystonia was made. The patient was started on baclofen and clonazepam with an option of botulinum toxin injection in case a poor response to the above medication.

Figure 1: MRI brain FLAIR axial sections (A, B, D) and right parasagittal T2 section (C) showing atrophy with gliosis and encephalomalacia of right lateral medulla (A), pons, middle cerebellar peduncle (B) and right-postero-inferior predominant bilateral cerebellar hemisphere atrophy (A, C). Basal ganglia and thalamus appear normal (D)

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Discussion

This report describes a case of poststroke dystonia secondary to a cerebellar infarct with a long latency between the stroke and onset of dystonia. Poststroke dystonia is typically described following basal ganglia involvement; however, although rare, cerebellar^[1],[2] and brainstem^[3] strokes may also cause poststroke dystonia. In the present case the right pontomedullary junction and the right cerebellum were involved which can be implicated for the dystonia. The cerebellar lesion probably played a bigger role in this patient, as dystonia due to brainstem lesions are usually contralateral to the lesion whereas, dystonia is ipsilateral in the case of cerebellar lesions.^{[2],[3],[4],[5]} Midbrain lesions can cause ipsilateral dystonia if the superior cerebellar peduncle is involved before decussation.^[5],[6]

Typically, cerebellar strokes lead to axial dystonia in the form of oromandibular or cervical dystonia. To the best of our knowledge, there are only two cases of UL dystonia secondary to isolated cerebellar stroke, either due to infarct or hemorrhage.^[2],[7] In addition, there are a few case reports of ipsilateral dystonia secondary to cerebellar lesions other than stroke, such as tubercular abscess and tumor.^[2] Somatotopically, the UL is widely represented in the superior as well as inferior posterior cerebellar hemisphere. The lesions in the present case involved these regions and could explain the observed dystonia.^[2] Several animal and human studies have shown the role of the cerebellum in both primary and secondary dystonia.^[2] Widespread cerebellar dysfunction leads to abnormal postures involving multiple body parts, whereas, limited dysfunction can cause focal or segmental dystonia as observed our case.^[8] In addition, various physiological studies have been performed to investigate role of the cerebellum in dystonia as a network disorder.^[9] A normal transient reduction in excitability of the hand area on transcranial magnetic stimulation of the cerebellum is reduced or abolished in patients with focal hand dystonia.^[10] This finding was consistent with the positron emission tomography studies which showed disruption of the cerebella–thalamic–cortical pathway. Furthermore, eye blink classical conditioning which is processed by the cerebellar cortex is impaired in cervical dystonia but was normal in secondary dystonia and in genetic dystonia.^[10]

Poststroke dystonia usually develops within a year after the stroke, and in contrast, the present case had a long latency of 9 years. However, this latency may be variable and ranges from 1 month to more than 15 years in previous reports.^[11],[12] The pathophysiological mechanism of delayed onset progressive movement disorders are poorly understood. It has been postulated that unbalanced motor recovery due to slowly evolving aberrant neuronal regeneration and reorganization which leads to the development of a pathological pathway in a susceptible individual may cause delayed onset and progression.^{[1],[11],[12]} In the present case, despite the high probability of dystonia being secondary to the cerebellar stroke, we cannot entirely rule out the possibility of a idiopathic isolated dystonia with or without a genetic cause as we did not perform genetic analysis in the patient. Genetic dystonia due to mutations in ANO3, GNAL, CACNA1B can present with adult-onset isolated focal dystonia akin to our case.^[13] In addition, there was bilateral cerebellar atrophy that cannot be completely explained by a unilateral stroke. Even though bilateral cerebellar changes have been observed following a unilateral cerebellar lesion,^[14] the possibility of an additional degenerative disease cannot be entirely ruled out. However, there were no ocular, axial or appendicular cerebellar symptoms or signs suggestive of degenerative cerebellar disorder except for a mild impairment of tandem gait. A functional imaging of the brain would have provided more information regarding the role of the cerebellum in the dystonia, but was not performed in this patient.

In conclusion, cervicobrachial segmental dystonia can be a sequela of cerebellar stroke and may occur even after a prolonged latency. This should be considered in a case of segmental dystonia with the past history of cerebellar stroke.

Author contributions

Vikram V. Holla, Sudhakar Pushpa Chaithra contributed in acquisition and interpretation of data and in writing first draft of the manuscript. Shweta Prasad and Pramod Kumar Pal contributed in interpretation of data and in review and critique of the manuscript.

Ethical compliance statement

Ethical approval for this study was waived by the institute ethics committee owing to the retrospective nature of the study. Written informed consent of the patient was obtained for the video recording and for online publication and dissemination.

Conflicts of interest

There are no conflicts of interest.

Financial support and sponsorship

Nil.

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