A 38-year-old woman presented with cervical dystonia in the context of a recent surgery to remove a vestibular schwannoma. She initially presented to neurology with pain in the right arm, and MRI of the brain showed an incidental right-sided vestibular schwannoma (Video 1, Segment 1). An elective gamma-knife procedure was performed, which failed. Hydrocephalus requiring ventriculoperitoneal shunt insertion developed, and 3 years following the initial procedure the lesion was surgically excised. Surgery was further complicated by right middle cerebellar peduncle injury, extending to the cerebellopontine angle and marginally to the right pontine tegmentum, with subsequent mass effect on cerebellum displayed on follow-up MRI (Video 1, Segment 2). Six months later, the patient experienced forced head deviation to the right, with difficulty moving from this position. Examination revealed clear right-sided torticollis, with hypertrophy of the left sternocleidomastoid muscle. Cervical dystonia worsened with action and nearly resolved with the patient lying down. A clear , where symptoms improved with the patient touching the side of her head, was present (Video 1, Segment 3). Findings consistent with injury to the cerebellar pathways were additionally exhibited. She demonstrated clear dysarthria, bilateral dysmetria, dysdiadochokinesia (worse on the right), and prominent gait ataxia (Video 1, Segment 4). Although a possible role of the schwannoma itself in the cervical dystonia pathogenesis cannot be entirely ruled out, the timing of signs, occurring soon after the postsurgical injury, suggest a prominent involvement of structures lying within the cerebellar pontine angle.
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http://dx.doi.org/10.1002/mdc3.12674 | DOI Listing |
J Voice
January 2025
Department of Otolaryngology-Head and Neck Surgery, UCSF Voice and Swallowing Center, UCSF School of Medicine, San Francisco, CA. Electronic address:
Background: Laryngeal respiratory dystonia (LRD) is diagnosed based on clinical presentation, patient history, and physical examination. Key indicators include dyspnea, desynchronized breathing patterns, and laryngoscopic findings that reveal vocal fold adduction during inspiration. Treatment for LRD remains controversial and often yields limited effectiveness.
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January 2025
Center for Brain Circuit Therapeutics, Department of Neurology, Brigham & Women's Hospital, Harvard Medical School, Boston, MA 02115.
Deep brain stimulation is an efficacious treatment for dystonia. While the internal pallidum serves as the primary target, recently, stimulation of the subthalamic nucleus (STN) has been investigated. However, optimal targeting within this structure and its surroundings have not been studied in depth.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Neurology, Joondalup Health Campus, Joondalup, Western Australia, Australia.
Anti-dipeptidyl-peptidase-like protein 6 antibody-mediated disease is a rare autoimmune encephalitis typically presenting with diarrhoea and/or weight loss, central nervous system hyperexcitability and cognitive dysfunction. We present a case of a young woman with 10 days of diplopia and unsteadiness in the context of dysthymia and significant weight loss over 2 months. Initial examination demonstrated mixed dysconjugate nystagmus and ataxic gait.
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Department of Orthopedic Surgery, Fujimi-Kogen Hospital, Fujimi-Kogen Medical Center, Fujimi, JPN.
This study reports on an extremely rare case of non-traumatic atlantoaxial rotatory fixation (AARF) in an adult. Although there are numerous reports on traumatic AARF in adults, those on non-traumatic AARFs are limited. We present the case of a 25-year-old woman who developed neck pain with a limited range of motion (ROM) that began upon waking without any particular inducement.
View Article and Find Full Text PDFJ AAPOS
December 2024
Department of Ophthalmology, Boston Children's Hospital, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts; Picower Institute for Learning and Memory, Massachusetts Institute of Technology, Cambridge, Massachusetts. Electronic address:
Ocular torticollis is traditionally attributed to eye misalignment, nystagmus, ptosis, or refractive error. We present 3 pediatric cases of acquired torticollis caused by a foreign body beneath the upper eyelid. The head posturing presumably developed to minimize contact of the foreign body with the corneal surface and mitigate ocular discomfort.
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