Progressive quadriparesis and inflammation: A common disease, a rare presentation.

A previously healthy, 10-years-old girl presented with progressively worsening pain and weakness of the limbs for the past 2 weeks. It initially started with low-grade fever lasting for 4 days followed by severe pain over bilateral lower and upper limbs. Gradually she became bed-ridden. On examination, she had severe neck rigidity, generalized tenderness all over the body, generalized hyperalgesia, hyporeflexia, bilateral extensor plantar response and toe-walking. An initial clinical diagnosis of Landry-Guillain Barry syndrome was considered. Nerve conduction study showed generalized, demyelinating polyneuropathy. She was administered IVIG and was evaluated for other causes of arachnoiditis. MRI brain and spine showed enhancement and clumping of nerve roots in the conus and cauda equina. CECT chest showed necrotic mediastinal lymphnodes. A final diagnosis of disseminated tuberculosis with tuberculous arachnoiditis was considered and she was administered ATT, pulse methylprednisolone followed by maintenance oral corticosteroids. Currently, after 5 months of therapy, she has recovered clinically.

Lumbar arachnoiditis: Does imaging associate with clinical features?

OBJECTIVES: Lumbar arachnoiditis is a rare and debilitating neurologic disorder with multiple etiologies and a spectrum of imaging and clinical characteristics. Prior reports have anecdotally claimed that no association exists between findings of arachnoiditis observed on magnetic resonance imaging (MRI) and those assessed clinically. The purpose of this study was to determine if MRI features of lumbar arachnoiditis associate with the clinical findings of the disorder. PATIENTS AND METHODS: Twenty eight patients with lumbar arachnoiditis reported on MRI between 2012 and 2018 were retrospectively identified. A variety of MRI and clinical features of lumbar arachnoiditis were cataloged for these patients based on common findings discovered through literature review. Imaging findings included cauda equina nerve root contour and thickening, adhesion location, level of involvement, enhancement, and Delamarter group. Clinical findings included demographics, etiology, symptom dynamics, and signs/symptoms. Fisher's exact tests were used to determine associations between the imaging and clinical features of lumbar arachnoiditis. RESULTS: In general, MRI findings did not associate with the clinical features of lumbar arachnoiditis with a few exceptions. Most notably, confounding lumbar pathology was associated with symptom dynamics (p = 0.004) and nerve root contour was associated with motor and sensory symptoms (p = 0.01). The suspected arachnoiditis etiology of the majority of patients was either post-operative or post-infectious in nature. CONCLUSION: MRI findings in lumbar arachnoiditis offer limited insight into the clinical presentation of the disorder.

Longitudinal measurements of syrinx size in a rat model of posttraumatic syringomyelia.

OBJECTIVE Syringomyelia pathophysiology is commonly studied using rodent models. However, in vivo studies of posttraumatic syringomyelia have been limited by the size of animals and lack of reliable noninvasive evaluation techniques. Imaging the rat spinal cord is particularly challenging because the spinal cord diameter is approximately 1-3 mm, and pathological lesions within the spinal cord parenchyma are even smaller. The standard technique has been histological evaluation, but this has its limitations. The aim of the present study was to determine whether syrinx size could be reliably measured using a preclinical high-field MRI animal system in a rat model of posttraumatic syringomyelia. METHODS The authors used an existing rat model of posttraumatic syringomyelia, which was created using a controlled pneumatic compression device to produce the initial spinal cord injury, followed by a subarachnoid injection of kaolin to produce arachnoiditis. T2-weighted MRI was performed on each animal using a 9.4-T scanner at 7, 10, and 13 weeks after injury. Animals were killed and syrinx sizes were calculated from in vivo MRI and histological studies. RESULTS MRI measurements of syrinx volume and length were closely correlated to histological measurements across all time points (Pearson product moment correlation coefficient r = ± 0.93 and 0.79, respectively). CONCLUSIONS This study demonstrates that high-field T2-weighted MRI can be used to measure syrinx size, and data correlate well with syrinx size measured using histological methods. Preclinical MRI may be a valuable noninvasive technique for tracking syrinx formation and enlargement in animal models of syringomyelia.

Symptomatic spinal cord kinking due to focal adhesive arachnoiditis, with ossification of the ligamentum flavum: a case report.

STUDY DESIGN: A case report. OBJECTIVE: To describe a rare case of symptomatic spinal cord kinking due to focal adhesive arachnoiditis, with ossification of the ligamentum flavum (OLF). SUMMARY OF BACKGROUND DATA: Spinal cord kinking without spinal surgery is rare, and symptomatic spinal cord kinking due to focal adhesive arachnoiditis, with OLF is even rarer. METHODS: A 66-year-old female presented with numbness of the lower extremities and subsequently experienced gait disturbance due to motor weakness. Magnetic resonance imaging of the thoracic spine showed anterior displacement and kinking of the spinal cord from T11 to T12. Laminectomy and OLF resection were performed. The arachnoid membrane at the affected part was markedly thick and seemed cloudy. Adhesiolysis for arachnoid adhesion and release of spinal kinking were performed. RESULTS: She could walk with a cane 6 months postoperatively. One year postoperatively, thoracic computed tomography-myelography showed that the cord was repositioned in the dural sac, and that release of the spinal cord kink was maintained. CONCLUSION: Symptomatic spinal cord kinking due to focal adhesive arachnoiditis, with OLF is a rare clinical condition. It was difficult to diagnose the precise pathology of the spinal cord before surgery. Microsurgical arachnoidolysis resolved the spinal cord kinking, and no recurrence was noted within the follow-up period. LEVEL OF EVIDENCE: N/A.

Arachnoiditis.

Questions from patients about pain conditions and analgesic pharmacotherapy and responses from authors are presented to help educate patients and make them more effective self-advocates. The topic addressed in this issue is arachnoiditis, its symptoms, and possible treatment approaches.

The relationship between localized subarachnoid inflammation and parenchymal pathophysiology after spinal cord injury.

Subarachnoid inflammation following spinal cord injury (SCI) can lead to the formation of localized subarachnoid scarring and the development of post-traumatic syringomyelia (PTS). While PTS is a devastating complication of SCI, its relative rarity (occurring symptomatically in about 5% of clinical cases), and lack of fundamental physiological insights, have led us to examine an animal model of traumatic SCI with induced arachnoiditis. We hypothesized that arachnoiditis associated with SCI would potentiate early parenchymal pathophysiology. To test this theory, we examined early spatial pathophysiology in four groups: (1) sham (non-injured controls), (2) arachnoiditis (intrathecal injection of kaolin), (3) SCI (35-g clip contusion/compression injury), and (4) PTS (intrathecal kaolin+SCI). Overall, there was greater parenchymal inflammation and scarring in the PTS group relative to the SCI group. This was demonstrated by significant increases in cytokine (IL-1α and IL-1ß) and chemokine (MCP-1, GRO/KC, and MIP-1α) production, MPO activity, blood-spinal cord barrier (BSCB) permeability, and MMP-9 activity. However, parenchymal inflammatory mediator production (acute IL-1α and IL-1ß, subacute chemokines), BSCB permeability, and fibrous scarring in the PTS group were larger than the sum of the SCI group and arachnoiditis group combined, suggesting that arachnoiditis does indeed potentiate parenchymal pathophysiology. Accordingly, these findings suggest that the development of arachnoiditis associated with SCI can lead to an exacerbation of the parenchymal injury, potentially impacting the outcome of this devastating condition.

Tuberculous optochiasmatic arachnoiditis: a devastating form of tuberculous meningitis.

Tuberculous meningitis is primarily a disease of the meninges of brain and spinal cord along with adjacent brain parenchyma. The characteristic pathological changes are meningeal inflammation, basal exudates, vasculitis and hydrocephalus. Tuberculous meningitis has a strong predilection for basal parts of the brain. Exudates, if dominantly present in the interpeduncular, suprasellar and Sylvian cisterns, result in optochiasmatic arachnoiditis and tuberculoma. Optochiasmatic arachnoiditis and tuberculoma are devastating forms of tuberculous meningitis and often associated with profound vision loss. This clinical entity more frequently affects young adults. In a recent study, on the multivariate logistic regression analysis, female sex, younger age and raised cerebrospinal fluid protein content were identified as predictors for developing optochiasmatic arachnoiditis. Frequently, optochiasmatic tuberculoma and optochiasmatic arachnoiditis develop paradoxically while a patient is being treated with anti-TB drugs. MRI reveals confluent enhancing lesions that are present in the interpeduncular fossa, pontine cistern, and the perimesencephalic and suprasellar cisterns. Management of tuberculous optochiasmatic arachnoiditis and optochiasmatic arachnoiditis tuberculoma has been variable. Treatment of optochiasmatic arachnoiditis continues to be a challenge and the response is generally unsatisfactory. In isolated case reports and in small series, corticosteroids, methyl prednisolone, thalidomide and hyaluronidase have been used with variable success. The benefit from neurosurgery is controversial and deterioration may follow the initial temporary improvement. Management of paradoxical optochiasmatic arachnoiditis is also controversial. Some patients regain vision following treatment with anti-TB drugs and continued usage of corticosteroids. Neurosurgery may be considered in the patients with either treatment failure or when diagnosis is in doubt. In conclusion, presence of optochiasmatic arachnoiditis or tuberculoma has important therapeutic and prognostic implications for patients of tuberculous meningitis.

Syringomyelia due to thoracic spinal stenosis with ossified ligamentum flavum--case report.

A 50-year-old male presented with back pain and numbness of the lower extremities persisting for 10 years. He had played volleyball for a long period until recently. He had no history of meningitis or traumatic injury. Magnetic resonance imaging revealed a syringomyelia located in the region from T8 to T9 without contrast enhancement or Chiari malformations. Computed tomography showed T9-10 spinal stenosis caused by the right enlarged ossified yellow ligament. Decompressive laminectomy was performed and the ossified ligament removed. Due to the finding of arachnoid thickening and adhesions during the intradural operation, shunting was also performed. Postoperatively, the neuroimaging and clinical findings improved. Syringomyelia is often associated with Chiari malformations, trauma, spinal tumor, hemorrhaging, and meningitis. We suggest that repeated minor mechanical damage caused by physical exercise in addition to long-standing compression of the spinal cord due to spinal spondylosis could induce severe arachnoid fibrotic change similar to adhesive arachnoiditis, which may be one of the main triggers of syringomyelia. Extradural decompressive surgery is considered to be the initial treatment for syringomyelia associated with spinal spondylosis.

Simplified aqueductal stenting for isolated fourth ventricle using a small-caliber flexible endoscope in a patient with neurococcidiomycosis: technical case report.

OBJECTIVE: Endoscopic aqueductoplasty and stenting are a preferred treatment for isolated fourth ventricle syndrome related to membranous aqueductal obstruction. We describe a technique using a small-caliber flexible endoscope that may address some limitations of current strategies. CLINICAL PRESENTATION: A 39-year-old woman with hydrocephalus caused by neurococcidiomycosis and a functional right frontal ventriculoperitoneal shunt presented with vomiting and an isolated fourth ventricle. Magnetic resonance imaging showed an enlarged fourth ventricle and exuberant basilar arachnoiditis obstructing the outlet foramina of the fourth ventricle. Ventriculography indicated aqueductal obstruction. INTERVENTION: Aqueductoplasty was planned to allow spinal fluid to flow from the fourth ventricle to the ventriculoperitoneal shunt. A stent-endoscope construct was prepared by feeding a flexible endoscope through a ventricular catheter cut 4 cm from the tip. The flexible endoscope was contoured to fit the anatomy of the aqueduct. Uncomplicated aqueductoplasty was performed through a single left frontal burr hole using the stent-endoscope construct to perforate a membranous veil and inspect the fourth ventricle. The stent was deployed over the endoscope using the proximal end of the catheter to deliver and secure the stent as the endoscope was withdrawn. CONCLUSION: Aqueductoplasty and stenting using a small-caliber flexible endoscope is feasible. The endoscope can be contoured to suit the anatomy of the aqueduct and improves visualization of the leading edge of the stent during deployment. Furthermore, when the endoscope is used to create the perforation, the target is not obscured by the shaft of the device used to make the perforation.

Lumbar arachnoiditis and thecaloscopy: brief review and proposed treatment algorithm.

The term arachnoiditis describes the inflammation of the meninges and subarachnoid spaces. Lumbar arachnoiditis is characterized by obliterated nerve root sleeves and the adherence of nerve roots to each other in the proximity of the cauda equina, and may be secondary to infectious diseases or tumors, iatrogenic (subsequent to spinal surgery) or idiopathic. It is not very clearly defined epidemiologically or clinically, and various theories regarding its pathophysiology have been proposed; furthermore, its treatment is difficult because there is a lack of evidence-based diagnostic and therapeutic gold standards. Thecaloscopy has been recently described as a novel technique for retrograde transcutaneous neuroendoscopic inspection of the subarachnoid structures of the lumbar thecal sac; it has also been suggested for the treatment of lumbar arachnoiditis. We here review the most modern techniques for the treatment of this disease such as thecaloscopy and neurostimulation.

Role of the blood-spinal cord barrier in posttraumatic syringomyelia.

OBJECT: Posttraumatic syringomyelia produces a significant burden of pain and neurological deficits in patients with spinal cord injury. The mechanism of syrinx formation is unknown and treatment is often ineffective. A possible explanation for syrinx formation is fluid leakage from the microcirculation in the presence of a compromised blood-spinal cord barrier (BSCB). The aim of this study was to investigate the structural and functional integrity of the BSCB in a model of posttraumatic syringomyelia. METHODS: The excitotoxic amino acid and arachnoiditis model of syringomyelia was used in 27 Sprague-Dawley rats. Structural integrity of the BSCB was assessed using immunoreactivity to endothelial barrier antigen (EBA), and loss of functional integrity was assessed by extravasation of intravascular horseradish peroxidase. Animals were studied after 3 days, or at 1, 3, 6, or 12 weeks after surgery. There were laminectomy-only and saline injection control animals for comparison at each time point. RESULTS: Syrinxes formed in 16 of the 17 animals injected with excitotoxic amino acid. Loss of structural and functional integrity of the BSCB in syrinx animals was noted at all time points. Disruption of the BSCB was most dramatic in tissue adjacent to the syrinx, and in the central and dorsal gray matter. Changes in EBA expression generally corresponded with altered vascular permeability, although in the acute stages, widespread vascular permeability occurred without a corresponding decrease in EBA expression. At the later time points (3-12 weeks) EBA expression was often absent, although no vascular leakage was observed. CONCLUSIONS: This study demonstrated a prolonged structural and functional disruption of the BSCB in this model of posttraumatic syringomyelia. Loss of functional integrity of the BSCB, with fluid entering the interstitial space of the spinal cord, may contribute to initial cyst formation after spinal cord injury and subsequent enlargement of the cyst, to produce posttraumatic syringomyelia.

[Pathomechanisms of spinal canal stenosis - upright MRI image gallery]. / Pathomechanismen der spinalen Enge - Upright-MRT-Bildergalerie.

AIM: Lumbar spinal canal stenosis is a common disease of the elderly patient, with a high prevalence and clinical importance. MRI is the established method of choice for the imaging of spinal canal stenosis. However, there is often a discrepancy between the clinical symptoms and the spinal canal stenosis as shown using MRI in a supine position. In such cases preoperative functional imaging is often warranted. METHODS: In an image gallery three cases of a functional spinal canal stenosis of the lumbar spine are shown. In all three patients a dynamic, positional MRI (upright MRI) was performed. RESULTS: The pathomechanisms of the spinal canal stenosis could be shown in all three cases. CONCLUSION: Using upright MRI a functional spinal canal stenosis can be shown. The pathomechanisms of the spinal canal stenosis are discussed. The possibilities and limitations of this new imaging modality are presented and analysed.

Tensile radial stress in the spinal cord related to arachnoiditis or tethering: a numerical model.

Spinal arachnoiditis comprises fibrous scarring of the subarachnoid space, following spinal trauma or inflammation, and is often associated with syringomyelia. We hypothesised that cord-to-dura attachments could cause transient tensile cord radial stress, as pressure waves propagate. This was tested in a fluid-structure interaction model, simulating three types of cord tethering, with 'arachnoiditis' confined to a short mid-section of the cord. The annular system was excited abdominally with a short transient, and the resulting Young and Lamb waves and reflections were analysed. Radial mid-section tethering was less significant than axial tethering, which gave rise to tensile radial stress locally when the cord was not fixed cranially. Simulated as inextensible string connections to the dura, arachnoiditis caused both localised tensile radial stress and localised low pressure in the cord as the transient passed. The extent of these effects was sensitive to the relative stiffness of the dura and cord. Tensile radial stress may create a syrinx in previously normal cord tissue, and transiently lowered pressure may draw in interstitial fluid, causing the syrinx to enlarge if fluid exit is inhibited. The suggested mechanism could also explain the juxtaposition of syrinxes to regions of arachnoiditis.

Papilledema as a manifestation of a spinal subdural abscess.

Papilledema is an uncommon presentation of spinal cord processes. Spinal subdural abscess (SSA) is a rare site of post-operative infection. We report a patient who developed papilledema as the primary manifestation of a post-operative lumbar subdural abscess. A spinal abscess should be considered in the post-operative spinal surgery patient who develops papilledema in the setting of persistent back pain. The increased intracranial pressure associated with lumbar spinal cord abscess most likely results from a markedly elevated cerebrospinal fluid (CSF) protein or the disruption of CSF flow in the spinal cul-de-sac.

Focal spinal arachnoiditis increases subarachnoid space pressure: a computational study.

BACKGROUND: Enlarging fluid filled cystic cavitations form within the spinal cord in up to 28% of spinal cord injured patients. These post-traumatic syrinxes can cause neurological deterioration and current treatment results are unsatisfactory. Localized scar tissue (arachnoiditis) within the subarachnoid space at the level of injury has been suggested to be involved in the pathogenesis of syrinx formation. This study tests the hypothesis that pressure pulses in the subarachnoid space are accentuated adjacent to regions of arachnoiditis, which may drive fluid into the spinal cord and contribute to syrinx formation. METHODS: An axisymmetric, cylindrical computational fluid dynamics model was developed to represent the subarachnoid space under normal physiological conditions and in the presence of arachnoiditis. Cerebrospinal fluid flow into the model was estimated from magnetic resonance imaging flow studies. Arachnoiditis was modelled as a porous obstruction in the subarachnoid space. FINDINGS: Peak fluid pressures were higher above the obstruction than in the absence of obstruction. The peak pressures were strongly dependent on the permeability of the obstruction. INTERPRETATION: Elevations in subarachnoid space pressures due to arachnoiditis may facilitate fluid flow into the spinal cord, enhancing syrinx formation. This suggests that it may be worthwhile to investigate strategies that inhibit arachnoiditis or minimize systolic pressure peaks for treating or preventing syringomyelia.

The prognosis of hearing impairment complicating HIV-negative cryptococcal meningitis.

Eight patients who had sensorineural hearing loss (SNHL) associated with cryptococcal meningitis were studied. After a minimum 3-year follow-up, one had died. Among the seven survivors, three had improved, two stabilized, and two progressed. Predictive factors included visual disturbance, meningeal enhancements on MRI, and a CSF cryptococcal antigen titer of >1:1,024. SNHL accounted for 30.8% (8/26) of cryptococcal meningitis patients in our study.

Subarachnoid pressure-dependent change in syrinx size in a patient with syringomyelia associated with adhesive arachnoiditis. Case report.

The pathophysiology of syringomyelia is still not well understood. Current prevailing theories involve the assumption that cerebrospinal fluid (CSF) flows into the syrinx from the subarachnoid space through the perivascular space of Virchow-Robin. Reported here is the case of a patient with syringomyelia in which this course is clearly contradicted. This patient with a holocord syrinx associated with adhesive arachnoiditis was treated 3 years previously with insertion of a subarachnoid-peritoneal shunt and had recently experienced worsening myelopathy. On surgical exploration, the shunt system was functioning normally. The medium-pressure shunt valve was replaced with an adjustable valve with a higher closing pressure setting, thus increasing the CSF pressure in the subarachnoid space. Contrary to prevailing theories, this procedure markedly reduced the size of the syrinx. This case provides direct evidence that the syrinx size is inversely related to subarachnoid CSF pressure and supports the hypothesis that the pressure gradient across the spinal cord parenchyma is the force that generates syringes in syringomyelia.

Theoretical analysis of the pathophysiology of syringomyelia associated with adhesive arachnoiditis.

OBJECTIVE: To apply a theoretical model to analyse the derangement of cerebrospinal fluid (CSF) dynamics in syringomyelia associated with adhesive arachnoiditis. METHODS: An electrical circuit model of CSF dynamics in the spine was used. With this model, the derangement of CSF dynamics in adhesive arachnoiditis was simulated. The effects of various surgical procedures were then analysed, such as syringo-subarachnoid shunting, subarachnoid bypass, and foramen magnum decompression. RESULTS: When CSF flow in the subarachnoid space was obstructed at a certain point, the pressure inside the spinal cord increased in the segment immediately distal to the blockage. This location of increased pressure corresponded to the preferred site of syrinx formation in adhesive arachnoiditis. Syringo-subarachnoid shunting, subarachnoid bypass, and foramen magnum decompression were all effective at reducing this pressure gradient. CONCLUSIONS: Blockage of the spinal subarachnoid CSF pathway produces a relative increase in the pressure inside the spinal cord distal to the blockage point. Repetitive formation of this pressure gradient then induces CSF leakage into the spinal parenchyma, leading to the formation of syringomyelia. Using this model, alternative surgical procedures could be suggested that might be effective in treating this disease.

Altered subarachnoid space compliance and fluid flow in an animal model of posttraumatic syringomyelia.

STUDY DESIGN: A histologic study of cerebrospinal fluid tracers in Sprague-Dawley rats undergoing lumboperitoneal shunt insertion in the excitotoxic animal model of posttraumatic syringomyelia (PTS). OBJECTIVES: To determine the effects of cerebrospinal fluid (CSF) diversion from the subarachnoid space on perivascular flow (PVS) and syrinx formation in posttraumatic syringomyelia. SUMMARY OF BACKGROUND DATA: In an animal model of PTS, fluid enters syringes from the subarachnoid space via perivascular spaces. Preferential PVS flow occurs at the level of the syrinx. It has been suggested that arachnoiditis predisposes to posttraumatic syringomyelia formation by obstructing subarachnoid cerebrospinal fluid flow and enhancing perivascular flow. MATERIALS AND METHODS: Thirty-two male Sprague-Dawley rats were investigated using the CSF tracer horseradish peroxidase (HRP), the excitotoxic model of PTS, and lumboperitoneal shunt insertion. Five experimental groups consisted of normal controls, syrinx only and shunt only controls, and shunt insertion before or after syrinx formation. In all groups except normal controls, CSF flow studies were performed 6 weeks after the final intervention. Grading scales were used to quantify HRP staining. RESULTS: All excitotoxic model animals formed syringes. Perivascular flow was greatest at the level of the syrinx. Cerebral cortex perivascular flow was significantly reduced after shunt insertion in animals with a syrinx (P < 0.05). Shunt insertion did not alter syrinx length or size. There were no significant differences between shunt and syrinx first groups. CONCLUSIONS: Increasing caudal subarachnoid space compliance with a shunt does not affect local CSF flow into the spinal cord and syrinx. These results suggest that localized alterations in compliance, as opposed to obstruction from traumatic arachnoiditis, may act as an important factor in syrinx pathogenesis.