Presyrinx consists of reversible spinal cord swelling without frank cavitation, as observed on T2 weighted magnetic resonance imaging (MRI). The condition may evolve into syringomyelia, but timely surgical interventions have achieved meaningful results. Here, we report the case of a 27-year-old woman who presented with headache, dizziness, and diplopia 2 months after suffering a mild head trauma. On MRI, hydrocephalus, downward herniation of the cerebellar tonsil, and a diffuse high signal change in the cervical spinal cord were detected. After insertion of a ventriculoperitoneal shunt, her neurological symptoms resolved, and she has had no signs of presyrinx recurrence for >4 years.
Adult ; Arnold-Chiari Malformation ; Cervical Cord ; Craniocerebral Trauma ; Diplopia ; Dizziness ; Female ; Headache ; Humans ; Hydrocephalus ; Magnetic Resonance Imaging ; Palatine Tonsil ; Recurrence ; Spinal Cord ; Syringomyelia ; Ventriculoperitoneal Shunt

STUDY DESIGN: Retrospective cohort study. PURPOSE: To investigate the correlation between the syrinx morphology and neuropathic pain caused by syringomyelia associated with Chiari I malformation. OVERVIEW OF LITERATURE: Neuropathic pain caused by syringomyelia is refractory and markedly impairs the patient. METHODS: We examined 24 patients with neuropathic pain caused by syringomyelia associated with Chiari I malformation. We statistically analyzed the illness duration and age at surgery between patients with and without neuropathic pain. Additionally, we classified the morphology of the syringes into deviated (D), enlarged (E), central (C), and bulkhead (B) types using T2-weighted axial imaging. Moreover, we investigated the correlation between syrinx morphology and neuropathic pain. A Mann–Whitney U-test was performed to compare between the presence or absence of neuropathic pain and the presence or absence of type D syringes. RESULTS: The median age at surgery was 27.5 years, and the median illness duration was 24 months. Among the 24 patients, 11 had preoperative neuropathic pain, one of which was free of neuropathic pain during the final follow-up period. Among patients with neuropathic pain, the syringes’ preoperative morphology was type D in nine patients and types E and C in one patient each. No patient exhibited type B morphology. Among patients without neuropathic pain, the preoperative morphology of the syringes was type D in three patients, type E in seven patients, and types C and B in two patients each. For types D and E, a correlation between neuropathic pain and syrinx morphology was observed. Moreover, type D was associated with significant neuropathic pain in both preoperative and postoperative states. CONCLUSIONS: This study showed a correlation between the morphological features of the syringes and the occurrence of neuropathic pain in patients with syringomyelia associated with Chiari I malformation.
Cohort Studies ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Neuralgia ; Retrospective Studies ; Syringes ; Syringomyelia

Syringomyelia associated with tuberculous meningitis is an extremely rare condition. Only a few studies have reported clinical experience with syringomyelia as a late complication of tuberculous meningitis. Twenty-six years after a tuberculous meningitis episode, a 44-year-old man presented with progressively worsening spastic paresis of the lower limbs and impaired urinary function for 2 years. Radiological examination revealed syringomyelia extending from the level of C2 to T9 and arachnoiditis with atrophy of the spinal cord between C2 and T3. We performed laminectomy from C7 to T1, dissected the arachnoid adhesion and placed a syringo-pleural shunt via keyhole myelotomy. One year after the operation, his neurological condition improved. The postoperative control magnetic resonance imaging revealed the correctly located shunt and significantly diminished syringomyelia cavities. We aim to discuss the mechanism of syrinx formation following tuberculous meningitis and to share our surgical therapeutic experience with this rare disease entity.
Adhesives* ; Adult ; Arachnoid* ; Arachnoiditis* ; Atrophy ; Cerebrospinal Fluid Shunts ; Humans ; Laminectomy ; Lower Extremity ; Magnetic Resonance Imaging ; Muscle Spasticity ; Paresis ; Rare Diseases ; Spinal Cord ; Syringomyelia* ; Tuberculosis, Meningeal*

Syringomyelia is a disorder in which a cavity has formed within the spinal cord. Idiopathic syringomyelia is not associated with identifiable causes such as Chari type 1 malformation, spinal cord tumor, vascular malformation, tethered cord, arachnoiditis, hydrocephalus, or previous spinal surgery. The main neurologic symptoms of idiopathic syringomyelia are toe-walking, constipation, incontinence, abnormal reflexes, and lower extremity weakness. Patients may present with various symptoms such as scoliosis, cutaneous markers, pain in the lower extremities or back, or may be asymptomatic. Herein, we report a young child with idiopathic syringomyelia presenting with subtle neck pain. A 23-month-old boy visited the neurologic clinic after 3 months of right occipital area neck pain. He had no history of trauma or central nervous system infection, and neurologic examination results were normal except for right posterior neck hyperesthesia. Brain and spinal magnetic resonance imaging showed an ovoid intramedullary cystic lesion (9.7×5.0×4.7 mm) at C6/7 of the spinal cord. There was no evidence of Chiari malformation or other lesions that can be primary pathologies of syringomyelia. Electromyogram/nerve conduction velocity results were normal. The subject was diagnosed as idiopathic syringomyelia. His symptoms and neurologic/radiologic indications showed no change at a 1-year follow-up. Idiopathic syringomyelia symptoms are varied and may be overlooked by physicians. Pediatricians may consider syringomyelia if patients complain about persistent sensory abnormality. All patients who present with syringomyelia should undergo detailed neuroimaging of the entire neuraxis to elucidate the proximate cause of the lesion.
Arachnoid ; Arachnoiditis ; Brain ; Central Nervous System Infections ; Child ; Constipation ; Follow-Up Studies ; Humans ; Hydrocephalus ; Hyperesthesia ; Infant ; Lower Extremity ; Magnetic Resonance Imaging ; Male* ; Neck ; Neck Pain ; Neuroimaging ; Neurologic Examination ; Neurologic Manifestations ; Pathology ; Reflex, Abnormal ; Scoliosis ; Spinal Cord ; Spinal Cord Neoplasms ; Syringomyelia ; Vascular Malformations

No abstract available.
Humans ; Neuralgia* ; Spinal Cord Stimulation* ; Spinal Cord* ; Syringomyelia*

Evidence suggested that glycogen synthase kinase-3β (GSK-3β) is involved in Nogo-66 inhibiting axonal regeneration in vitro, but its effect in vivo was poorly understood. We showed that stereotactic injection of shRNA GSK-3β-adeno associated virus (GSK-3β-AAV) diminished syringomyelia and promoted axonal regeneration after spinal cord injury (SCI), using stereotactic injection of shRNA GSK-3β-AAV (tested with Western blotting and RT-PCR) into the sensorimotor cortex of rats with SCI and by the detection of biotin dextran amine (BDA)-labeled axonal regeneration. We also determined the right position to inject into the sensorimotor cortex. Our findings consolidate the hypothesis that downregulation of GSK-3β promotes axonal regeneration after SCI.
Animals ; Axons ; drug effects ; metabolism ; Dependovirus ; genetics ; Glycogen Synthase Kinase 3 beta ; genetics ; metabolism ; Humans ; Nerve Regeneration ; genetics ; RNA, Small Interfering ; administration & dosage ; genetics ; Rats ; Sensorimotor Cortex ; drug effects ; pathology ; Spinal Cord Injuries ; genetics ; pathology ; therapy ; Syringomyelia ; genetics ; pathology ; therapy

Adult ; Electrocardiography ; Humans ; Male ; Myotonic Dystrophy ; complications ; pathology ; physiopathology ; Syringomyelia ; complications ; pathology ; physiopathology

OBJECTIVE: The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. METHODS: Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18–63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (≥50% decrease in the diameter of the syrinx) were investigated. The follow-up period was 72.7±55.6 months. RESULTS: Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. CONCLUSION: Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored.
Adult* ; Arachnoid ; Cerebrospinal Fluid ; Cisterna Magna ; Decompression ; Follow-Up Studies ; Foramen Magnum ; Humans ; Medulla Oblongata ; Rhombencephalon ; Syringomyelia

Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari's malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.
Decompression ; Encephalocele ; Foramen Magnum ; Humans ; Syringomyelia*

Neuropathic shoulder arthropathy or Charcot's shoulder is an extremely rare disease, and sometimes it is associated with cervical syringomyelia. Clinical symptoms of the disease include edema of the shoulder and restriction in range of motion. Radiological diagnosis can be made through plain radiography through a characteristic, atrophic destruction of the joint. We experienced a Charcot's joint of the shoulder wherein destruction of the joint progressed extremely quickly and reviewed the literature concerning this condition.
Arthropathy, Neurogenic ; Diagnosis ; Edema ; Joints ; Radiography ; Range of Motion, Articular ; Rare Diseases ; Shoulder* ; Syringomyelia*