Rare Cause Of Cervical Myelopathy Research Articles

Venous pathology targeted surgical management in Hirayama disease: A comprehensive case series of nine cases exploring this potential etiology.

Hirayama disease is a rare cause of cervical myelopathy predominantly affecting young individuals. The disease is classically characterized by muscle atrophy in the distal upper limbs. While various etiopathogenesis such as dural sac dysplasia, nerve root dysplasia, structural abnormalities of the spinal ligament, and venous dysplasia have been proposed, this study explores the potential role of venous pathology and surgical management on the basis of it. This is a prospective descriptive case series of nine cases. The diagnosis was made based on the Huashan diagnostic criteria which includes clinical manifestation, imaging, and electrophysiology. In cases where magnetic resonance imaging (MRI) failed to demonstrate engorged veins, a computed tomography (CT) venogram of the cervical spine was used as an imaging tool. All patients underwent cervical laminectomy and coagulation of the posterior epidural venous plexus with or without laminoplasty. All the patients were followed up regularly; clinical improvement and neck disability index were assessed. All nine patients were male and exhibited classical clinical features, electrophysiological abnormalities, and MRI findings except, in one patient where a CT venogram helped in establishing the diagnosis as the MRI was inconclusive. Postoperatively, all patients had neurological improvement and stabilization of the disease. All patients who underwent CT venogram and cervical spine X-ray in neutral and dynamic position demonstrated no recurrence of engorged venous plexus or significant instability except one patient developing kyphosis. One patient experiencing symptoms in the other limb underwent a second surgery. This comprehensive case series strongly supports venous pathology as a potential etiology of Hirayama disease. Surgical management with laminectomy and venous coagulation with or without expansile laminoplasty has delivered consistent improvement in neurological outcomes and long-term disease stabilization without the restriction of movements and lesser complications. However, further research is warranted to elucidate the mechanism underlying cervical venous dilatation.

Cervical Myelopathy Caused by a Ventriculoperitoneal Shunt: A Case Report

Intracranial hypotension is a rare cause of cervical myelopathy. Cerebrospinal fluid (CSF) shunt surgery, which belongs to the broader category of ventriculoperitoneal shunt surgery, is commonly chosen as the primary treatment option for hydrocephalus. However, such surgery can potentially lead to CSF overdrainage, resulting in the occurrence of intracranial hypotension. In the present case, spinal surgery was performed twice for cervical myelopathy; however, there was no improvement in symptoms such as gait disturbance. After the readjustment of the shunt valve pressure, the symptoms improved.

Lateral Suboccipital Craniotomy With C1-C2 Hemilaminectomies and C1-C3 Fusion for the Treatment of a C1-C2 Synovial Cyst Causing Spinal Cord Compression: 2-Dimensional Operative Video.

Atlantoaxial synovial cysts are a rare cause of cervical myelopathy. Here we describe a case of a 26-yr-old woman who presented with progressively decreasing right arm and leg strength and associated gait imbalance. On examination, she had diffuse weakness in the right upper and lower extremities, a positive right-sided Hoffman sign, and clonus in the right lower extremity. Computed tomography demonstrated an os odontoideum and a retro-odontoid cyst. Magnetic resonance imaging demonstrated a T1 hypointense, T2 hyperintense, slightly rim-enhancing retro-odontoid cyst causing a marked narrowing of the spinal canal, with resultant flattening and leftward deviation of the spinal cord. The patient consented to undergo cyst fenestration via a right suboccipital craniotomy and right C1-C2 hemilaminectomies, along with a C1-C3 instrumented posterior spinal fusion. This approach was chosen because it allows for cyst fenestration and instrumentation of the hypermobile cervical spine within the same incision. After the dura was opened and the arachnoid was dissected, the cyst was visualized compressing the spinal cord. The cyst was fenestrated just inferior to the C1 nerve rootlets, resulting in immediate egress of a gelatinous content; thereafter, all accessible cyst wall portions were removed. Fusion was performed with lateral mass screws at C1 and C3 and pars screws at C2. Pathological analysis described the cyst content as reactive fibrovascular tissue with cholesterol deposition. There were no complications associated with the procedure, and the patient's right-sided weakness had nearly resolved by postoperative day 1. Patient consent was granted for publication.

Isolated cervical spinal canal stenosis at C-1 in the pediatric population and in Williams syndrome

Isolated cervical canal stenosis at the level of the atlas (C-1) is a rare cause of cervical myelopathy in the pediatric population. It has been associated with several genetic disorders including spondyloepiphysial dysplasia congenita, Down syndrome, and Klippel-Feil syndrome. The purpose of this study is to highlight the authors' experience with 4 additional pediatric cases, review the literature, and report a new association of this disease with Williams syndrome. The medical records and radiological imaging studies of 4 patients treated at Texas Children's Hospital for symptomatic hypoplasia of the atlas were retrospectively reviewed. Pertinent patient demographic data, clinical presentation, imaging findings, and outcomes after surgery were recorded. A thorough literature review was performed, allowing the authors to compare and contrast their 4 cases to surgical cases already published in the literature. There were 11 boys and 1 girl in the aggregate series. The average age of the patients was 7 years (range 13 months-14 years), and the duration of symptoms prior to presentation was 6 months (range 0-36 months). The mean sagittal diameter of the spinal canal at the level of the atlas measured from the posterior aspect of the dens to the anterior aspect of the arch of C-1 was 11.9 mm (range 8.3-16 mm) in the aggregate series. In 2 new pediatric patients with hypoplasia of the atlas the disease was associated with Williams syndrome, which has not been previously described. Patients in the aggregate series were followed for an average of 18 months (range 3-50 months). Laminectomy of C-1 provided neurological improvement in all patients who presented. Isolated cervical spinal canal stenosis at the level of the atlas is a rare cause of cervical myelopathy. The authors hope that this report will prompt clinicians to consider it when searching for the origin of signs and symptoms of cervical myelopathy, especially in children.

C2/3 instability: unusual cause of cervical myelopathy in a child with Down syndrome

We report a rare cause of cervical myelopathy (CM) in a 10-year-old Down syndrome (DS) girl. She presented with progressive CM over 1year affecting her ability to ambulate or feed herself. The myelopathy was secondary to C2/3 instability. Surgical reduction and instrumented fusion have significantly improved her neurological status. This case emphasizes the importance of close follow-up of DS patients for early diagnosis and treatment of cervical spine abnormalities.

Cervical myelopathy due to chronic overshunting in a pediatric patient: case report and review of the literature

We present a rare cause of cervical myelopathy produced by an engorged suboccipital epidural venous plexus due to chronic cerebrospinal fluid (CSF) overdrainage. A 17-year-old boy with obstructive hydrocephalus due to a retrocerebellar cyst and secondary implantation of a ventricloperitoneal shunt (VP-shunt) presented with progressive spastic tetraparesis. MRI imaging revealed myelopathy due to significant compression of the cervical spinal cord by engorged epidural veins. Further assessment at a low-pressure setting revealed a broken shunt valve. The VP-shunt valve was changed with an additional anti-siphon device leading to a gradual increase of the intracranial pressure (ICP). After intensive physiotherapy, the patient showed slight clinical improvement. Follow-up imaging within nine days showed distinct regression of the dilated venous plexus at the cranial-cervical junction (CCJ) with the resolution of cord compression. Engorgement of the epidural venous plexus should always be considered in the differential diagnosis of myelopathy in long-term shunt patients even when classical clinical and radiological signs of overshunting are missing.

Cervical myelopathy from calcium pyrophosphate dihydrate crystal deposition: a case report

Calcification of the ligamentum flavum secondary to calcium pyrophosphate dihydrate (CPPD) deposition may be a rare cause of cervical myelopathy. We present a 56-year-old man with spinal stenosis secondary to CPPD disease and subacute cervical myelopathy following minor trauma. The patient had no history of CPPD disease. Posterior C4-C6 decompression and instrumented fusion were performed. Intraoperative findings were densely thickened ligamentum flavum and distortion of the cervical dura mater due to impingement. Histological sections of discs and ligamentum flavum specimens showed CPPD crystals. Postoperatively, neck pain and paresthesias improved immediately. At 2 weeks after spinal surgery, the patient experienced acute right knee swelling. Standard radiographs showed chondrocalcinosis of the knee menisci.

Cervical Myelopathy Due to a Hypoplastic Atlas

Congenital hypoplasia of the atlas is a rare cause of cervical myelopathy. We report a case of a 30-year-old man with cervical myelopathy caused by spinal stenosis at the level of the atlas associated with a hypoplastic but complete posterior arch of C1. Repetitive and persistent hyperextension causes micro- traumas at the level of spinal stenosis in the spinal cord. Hypoplasia of the atlas may result in serious neurologic manifestations and complications in time. In our case, the spinal canal diameter was calculated to be 11 mm at the level of the atlas on computerized tomography imaging. However, this diameter was 8 mm on magnetic resonance imaging owing to the thickened atlanto-occipital membrane. We suggest that the spinal canal diameter should be measured by magnetic resonance imaging. Treatment of developmental cervical steno- sis is surgical removal of the thickened posterior arc of the atlas and surrounding degenerative ligaments in symptomatic cases. The symptoms related to myelopathy may improve after surgery.

Cervical Myelopathy and Congenital Stenosis From Hypoplasia of the Atlas

Case reports of patients with cervical myelopathy to hypoplasia of the atlas. To report cases of cervical myelopathy due to congenital hypoplasia of the atlas and to review the literature. Six previously documented cases of congenital hypoplasia of the atlas as a cause of cervical myelopathy are reported in the literature. Three patient's clinical record and radiologic imaging studies as well as a thorough literature search are reported. Plain radiographs, computed tomography scans, magnetic resonance images, as well as somatosensory-evoked potential changes are displayed. Cervical myelopathy developed in three patients who were found to have congenital hypoplasia of the atlas. Laminectomy of C1 provided neurologic improvement in all three patients presented. Congenital hypoplasia of the atlas is a rare cause of cervical myelopathy. This report should broaden the radiographic differential diagnosis when seeking an explanation for the signs and symptoms of cervical myelopathy.

The Syndrome of Central Cervical Soft Disk Herniation

Central herniation of soft disk material into the ventral epidural space is a relatively rare cause of cervical myelopathy. A history of significant antecedent trauma or pain is singularly absent in this syndrome. Spastic ataxia of gait and impaired pain and touch sensibility are the common neurological abnormalities. Typically, the myelopathy develops in the presence of a narrowed cervical spinal canal. The myelographic picture is diagnostic but may be misleading as to the exact level of the herniation. Anterior discectomy and fusion have given excellent relief of symptoms in this group of patients.