Cervical and axial dystonia in a patient with syringomyelia

Silva‐Júnior, Francisco Pereira da; Santos, Jasper Guimarães; Sekeff-Sallem, Flávio Augusto; Lucato, Leandro Tavares; Barbosa, Egberto Reis

doi:10.1590/s0004-282x2012000900018

Cited by 7 publications

(6 citation statements)

References 5 publications

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“…Involuntary movements are a rare initial presentation of syringomyelia. Only a few studies reporting involuntary movements are currently available in the literature, and only 1 study reported posttraumatic involuntary movements [7‐9]. Myoclonus, tremor, and dystonia were the most commonly presented symptoms in a previous review [10].…”

Section: Discussionmentioning

confidence: 99%

Rhythmic Neck Muscle Spasms and Upper Limb Muscle Myoclonic Jerks as an Unusual Initial Presentation of Posttraumatic Cervicothoracic Syringomyelia: A Case Report

Lin

Tsai

2018

PM&R

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Posttraumatic syringomyelia with an initial presentation of involuntary movement is rare. We describe a 25-year-old patient who sustained complete traumatic spinal cord injury at the thoracic level and presented with rhythmic neck muscle spasms and upper limb muscle myoclonic jerks 1 month after trauma. Magnetic resonance imaging revealed syrinx formation between C3 and T1. Lumbar-peritoneal shunt and decompression were performed. The symptoms completely disappeared after surgery. This report highlights that rhythmic neck muscle spasms and upper limb muscle myoclonic jerks can be the initial and only manifestations of syringomyelia. LEVEL OF EVIDENCE: V.

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Section: Discussionmentioning

confidence: 99%

Rhythmic Neck Muscle Spasms and Upper Limb Muscle Myoclonic Jerks as an Unusual Initial Presentation of Posttraumatic Cervicothoracic Syringomyelia: A Case Report

Lin

Tsai

2018

PM&R

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“…Although the dog did have concurrent CM and syringomyelia, which has been associated with a variety of involuntary movements, we felt the patient’s phenotype was inconsistent with segmental spinal myoclonus or propriospinal myoclonus. We did consider that the patient’s signs could represent a variant of syringomyelic dystonia, which has not been previously reported in dogs [12, 13]. CM is the most common cause of syringomyelia in dogs, but may be an incidental finding in many at risk breeds [14, 15].…”

Section: Discussionmentioning

confidence: 99%

A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies

Pancotto

Rossmeisl

2017

J Clin Mov Disord

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BackgroundThe stiff person syndrome (SPS) is a rare and debilitating autoimmune disorder with an unknown pathogenesis and variable clinical presentation that can present a diagnostic challenge. Although entities that clinically mimic stiff-person spectrum disorders (SPSD) have manifested in horses, they have not been reported in dogs.Case presentationWe describe a 2-year-old beagle dog presented for progressive attacks of muscular rigidity and lordosis with superimposed spasms of the appendicular muscles triggered by tactile stimulation which resulted in marked gait impairment. Resting electromyography revealed continuous motor unit activity in the axial musculature. Compared to age-matched healthy beagle dogs, this patient had elevated glutamic acid decarboxylase antibody concentrations in serum and cerebrospinal fluid.ConclusionThis dog presented with phenotypic, electrodiagnostic, and immunologic criterion consistent with an SPSD, including elevated anti-GAD antibody titers, which we have termed the “stiff dog syndrome (SDS)”. Durable clinical improvement was achieved with symptomatic and immunosuppressive treatments including baclofen, gabapentin, prednisone, and intravenous immunoglobulin.Electronic supplementary materialThe online version of this article (doi:10.1186/s40734-017-0053-3) contains supplementary material, which is available to authorized users.

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“…In the literature, mostly in the form of case studies, movement disorders due to cervical cord lesions were also reported in syringomyelia (16), traumatic cervical spinal cord damage (12,17), cervical cord tumor (18) in addition to demyelinating diseases. The demyelination plaques on the cervical spinal cord in the three MS cases presented here suggested that the dystonias may likely be due to the damage of the somatosensory pathways of the spinal cord.…”

Section: Discussionmentioning

confidence: 99%

Focal Hand Dystonia as a Sign of Demyelinating Attack in Multipl Sclerosis: 'Report of Three Cases’

Öcek¹,

Köşkderelioğlu²,

Gücüyener³

et al. 2014

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Re cei ved/Ge lifl Ta ri hi: 28.09.2013 Ac cep ted/Ka bul Ta ri hi: 06.02.2014 Although it is known that dystonia is a basal ganglia disease, dystonic symptoms have been observed in association with lesions of various sites located in sensory and motor pathways. We report three cases of paroxysmal focal hand dystonia, which may be due to the damage of the somatosensorial pathways in the cervical spinal cord. We suggest that the dystonia in our patients may be related to these active demyelinating cervical plaques. Two female and one male patients with definite relapsing remitting Multiple sclerosis (MS) between the ages of 22 to 45 were admitted with serious disability while using their right hands. In all three cases abnormal posture in the right hand and involuntary sustained contractions together with minor choreiform movements of the fingers were observed. Cervical MRI showed contrast-enhancing demyelinating lesions at the level of C2-3 in all patients. One of the patient's cranial MRI revealed also two new contrast-enhancing plaques on the neighbourhood of right posterior lateral ventricle and parietal cortex. No new or enhancing lesion was detected in the basal ganglia; indicating that the cervical spinal cord lesions were responsible for hand dystonia. In one of the patients, the right median somatosensory evoked potential response was absent in accordance with the clinical symptom. All three patients were treated with 1 gr. intravenous methylprednisolone per day for 5-10 days. Approximately one month later, clinical symptoms have been completely disappeared and control cervical MRI revealed resolution of the active lesions in all.

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Cervical and axial dystonia in a patient with syringomyelia

Cited by 7 publications

References 5 publications

Rhythmic Neck Muscle Spasms and Upper Limb Muscle Myoclonic Jerks as an Unusual Initial Presentation of Posttraumatic Cervicothoracic Syringomyelia: A Case Report

Rhythmic Neck Muscle Spasms and Upper Limb Muscle Myoclonic Jerks as an Unusual Initial Presentation of Posttraumatic Cervicothoracic Syringomyelia: A Case Report

A case of stiff dog syndrome associated with anti-glutamic acid decarboxylase antibodies

Focal Hand Dystonia as a Sign of Demyelinating Attack in Multipl Sclerosis: 'Report of Three Cases’

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