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Spinal Cord Disease
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Structural spinal cord abnormality: Arnold–Chiari malformation with or without syringomyelia.Cervical or lumbar spondylosis.Tethered cord syndrome.Tumor of the spinal cord.AVM of the spinal cord.Granuloma (e.g. tuberculous) involving vertebrae and spinal cord.
Tethered Cord Syndrome
Published in Jacques Corcos, Gilles Karsenty, Thomas Kessler, David Ginsberg, Essentials of the Adult Neurogenic Bladder, 2020
Nishant Garg, Yahir Santiago-Lastra
Tethered cord syndrome (TCS) is a functional disorder of the spinal cord caused by pathologic anchoring of its caudal end, causing neurologic dysfunction.1 While there was originally disagreement within the neurosurgical community regarding the existence of TCS as a separate entity from other neurologic conditions, it is now recognized as a distinct disorder. Despite being a distinct pathologic condition with its own clinical manifestations, TCS has been observed to need subsequent treatment in 20%–50% of children with spina bifida, highlighting the interrelated nature of lumbar cord disorders.2
The Tethered Spinal Cord: Its Protean Manifestations, Diagnosis, and Surgical Correction
Published in Alexander R. Vaccaro, Charles G. Fisher, Jefferson R. Wilson, 50 Landmark Papers, 2018
Arjun V. Pendharkar, Raphael Guzman, Samuel H. Cheshier
The clinical entity of tethered cord has evolved significantly over the past 100 years. Several early reports had described a progressive neurological deficit in the context of a fixed spinal cord, but varying theories implicated thickened filum terminale, lipoma, myelomeningocele, and other associated pathologies. The present study aimed to further characterize the tethered cord syndrome in the absence of other obvious spinal dysraphisms and describe the clinical outcomes after laminectomy and surgical sectioning of the filum terminale.
Effect of untethering on occult tethered cord syndrome: a systematic review
Published in British Journal of Neurosurgery, 2022
Hamid Rezaee, Ehsan Keykhosravi
Tethered cord syndrome (TCS) is defined as a stretch-induced functional disorder of the spinal cord in which the caudal part of the spine is anchored by a deformed structure. It is associated with various underlying conditions, including open and closed forms of spinal dysraphism and more subtle structural abnormalities.1 Voiding dysfunction, lower-extremity weakness, progressive motor and sensory changes in the legs, back and leg pain, gait disturbance, orthopedic foot deformities, and scoliosis and/or urinary dysfunction are the main symptoms of TCS. The TCS appears in various forms, including tight filum terminale, lipomeningomyelocele, split cord malformations (diastematomyelia), dermal sinus tracts, and dermoids.2 Lumbosacral TCS is treated by surgical untethering via the section of the filum terminale (SFT).3
Absent sural responses in tethered cord syndrome
Published in The Journal of Spinal Cord Medicine, 2021
Elia G. Malek, Johnny Salameh, Nour Estaitieh, Achraf Makki
Tethered cord syndrome (TCS) is a progressive spinal cord disorder caused by stretch-induced dysfunction of the conus medullaris. Clinical signs and symptoms of TCS may be cutaneous, neurologic, musculoskeletal, and/or genitourinary.1 Toddlers and adolescents tend to present with gait or running difficulties due to progressive motor dysfunction, sensory deficits, progression of scoliosis, or development of orthopedic foot deformities.2 TCS may be associated with myelomeningocele, syringomyelia, diastematomyelia, dermoid sinus and intra or extradural lipoma which may exacerbate the clinical findings.3 Spine MRI demonstrates elongation and caudal descent of the conus medullaris (below L2 vertebral level) and a fatty filum terminale (usually >3 mm in diameter).4 While electrophysiological evaluation for patients with TCS was just limited to intraoperative monitoring, few studies performed a detailed electrophysiological assessment in non-operated patients.5,6 We report two pediatric cases with asymmetrically absent sural nerve response prior to their diagnosis of TCS.
First report of tethered cord syndrome in a patient with Verheij syndrome
Published in Ophthalmic Genetics, 2023
A. Kocaaga, S. Yimenicioglu, Y. Özdemir Atikel, O. Özkale Yavuz
All reported patients had mild to moderate global developmental delays. Other neurological symptoms of VRJS include seizures, Bell’s palsy, and autistic behaviors (6). The brain MRI of patients showed periventricular leukomalacia, partial agenesis of the corpus callosum, and white matter abnormalities (10). Brain MRI showed left hypoplastic bulbus oculi. The patients with VRJS had skeletal and vertebral abnormalities, such as scoliosis, vertebral fusion, hemivertebra, spina bifida, sacral dysplasia, coccyx agenesis, and pectus excavatum (3,5). We described scoliosis, a fused vertebra, and a tethered cord in the present case. However, there was only one VRJS report associated with a tethered spinal cord in the literature. Dauber et al. reported a female patient who had tethered cord-inducing severe scoliosis and a de novo c.505C>T variant in PUF60 (2). Tethered cord syndrome is a diverse clinical entity resulting from abnormal development of the neuraxis in which the medullary cone is fixed at a level lower than normal (10,11). Common clinical manifestations of a tethered spinal cord include lower-extremity weakness, leg or lowback pain, neurogenic bladder, and incontinence (12–14). We noted that tethered cord syndrome was associated with neurogenic bladder and urinary incontinence in the index case. Timely diagnosis of a tethered cord in VRJS syndrome will reduce possible morbidity and result in early surgical intervention of the patient. Therefore, tethered cord syndrome should be considered among the possible diagnoses, especially in patients with Verheij syndrome who have neurogenic bladder and urinary and fecal incontinence. The ophthalmologic abnormalities of patients with VRJS include irido-retinal or chorio-retinal coloboma, microphthalmia, strabismus, nystagmus and amblyopia (2,6,7). The ophthalmologic examination of our patient revealed bilateral microphthalmia and coloboma of the irido-choroidal, convergent strabismus, and bilateral horizontal nystagmus.