Spinal adhesive arachnoiditis in an adult patient with spinal muscular atrophy type 3 treated with intrathecal therapy.

BACKGROUND: Spinal adhesive arachnoiditis is a chronic inflammatory process of the leptomeninges and intrathecal neural elements. The possible causes of arachnoiditis are: infections, injuries of spinal cord, surgical procedures and intrathecal administration of therapeutic substances or contrast. CASE PRESENTATION: We present a case of 56-old woman with spinal muscular atrophy type 3 who developed a severe back pain in the lumbosacral region after the fifth dose of nusinersen given intrathecally. Magnetic resonance of lumbosacral spine showed spinal adhesive arachnoiditis. She received high doses of methylprednisolone intravenously, and later non-steroidal anti-inflammatory drugs, alpha lipoic acid, vitamins and rehabilitation with slight improvement. CONCLUSIONS: The authors summarize that scheduled resonance imaging of the lumbosacral spine may be an important element of the algorithm in the monitoring of novel, intrathecal therapy in patients with spinal muscular atrophy.

Intracranial Arachnoiditis and Hydrocephalus.

A 62-year-old man presented a diffuse and predominantly cisternal acute nonaneurysmal subarachnoid hemorrhage associated with hydrocephalus. An external ventricular drain was placed, followed by clinical deterioration after its removal. At this point, a heavily T2-weighted high-resolution sequence of a brain magnetic resonance imaging showed acute hydrocephalus recrudescence and multiple arachnoid adhesions in the supravermian and interpeduncular cisterns, creating a loculated/cystic appearance. The diagnosis of intracranial arachnoiditis was made. Intracranial arachnoiditis results from meningeal inflammation. Fibrosis and adhesions at the subarachnoid spaces may follow, restricting cerebrospinal fluid circulation, particularly at the cranial base. Hydrocephalus probably resulted from the combination of subarachnoid hemorrhage and extensive scaring at the basal cisterns, precluding transdural and transvenous cerebrospinal fluid efflux. Heavily T2-weighted high-resolution magnetic resonance imaging sequences allow an exquisite depiction of arachnoiditis, displaying obstructive cisternal membranes, and contribute to better etiologic assessment and management of hydrocephalus.

Patterns of Intrathecal Ossification in Arachnoiditis Ossificans: A Retrospective Case Series.

Arachnoiditis ossificans is an uncommon end-stage appearance of chronic adhesive arachnoiditis. Imaging features of arachnoiditis ossificans are characteristic and should be diagnosed to avoid unnecessary intervention and guide prognosis and management. In this case series, we retrospectively analyzed CT and MR imaging of 41 patients to identify common patterns of intrathecal ossification and present the common etiologies. Thirty-two patients had a confirmed history of spinal instrumentation, 7 were discovered on imaging without prior surgical history, 1 had a history of ankylosing spondylitis, and 1 had trauma. The most frequent site of ossification was at the conus and cauda equina. Four patterns of ossification were identified, including central, nerve root encasing, weblike, and peripheral. Arachnoiditis ossificans is an important, likely under-recognized consideration in patients who present with back pain. Diagnosis can be made readily on CT; MR imaging diagnosis is also possible but may be challenging.

Thoracal arachnoiditis ossificans associated with multifocal motor neuropathy: a case report.

The association of arachnoiditis ossificans with acquired peripheral nerve disease is rare. We report a case who presented with progressive myelopathy due to arachnoiditis ossificans from prior trauma, complicated with multifocal motor neuropathy. Intradural bone was removed at surgery.

"PTFE Sleeve Graft" Technique to Remove Neurovascular Conflict in Micro Vascular Decompression for Trigeminal Neuralgia.

Background: Microvascular decompression (MVD) of the trigeminal nerve is a well-accepted nondestructive procedure for trigeminal neuralgia. Usually, Teflon (PTFE) puff or felt graft techniques, which are most commonly used, are associated with arachnoiditis and recurrence among other complications. We use the "sleeve graft" technique using PTFE to separate the neurovascular conflict and here we describe our experience with the same in 376 cases. Objectives: To study the outcomes in 376 patients treated with sleeve graft technique for trigeminal neuralgia. Materials and Methods: For a period of 18 years, from 2002 to 2020, all cases of medically refractory trigeminal neuralgia were subjected to the "sleeve graft" technique for MVD. Pre- and post-operatively, pain score was given according to Barrow Neurological Institute pain intensity score. Cases were observed for any complications and pain relief in short and long-term follow-up. Results: In total, 376 cases of refractory primary trigeminal neuralgia cases, among which 198 patients underwent MVD with no prior intervention, 158 underwent MVD following percutaneous ablative procedure, 13 were "Revision MVD" previously done at other centers, and four were post gamma knife failure. There was no incidence of arachnoiditis or recurrence of symptoms. Further, 368 (97.8%) patients had complete recovery from symptoms while eight (2.2%) had partial recovery after 5 years of follow-up. Complications included hearing loss (n = 1), temporary hypoesthesia (n = 45), and permanent hypoesthesia (n = 7). Conclusion: "PTFE Sleeve Graft" technique to remove the neurovascular conflict in micro vascular decompression (MVD) for trigeminal neuralgia is a safe and effective technique that yields better results.

Cyclophosphamide therapy as an adjunct in refractory post-tubercular arachnoiditis.

INTRODUCTION: There is no satisfactory treatment for post tubercular arachnoiditis (TB arachnoiditis). We did this study to investigate the efficacy and safety of cyclophosphamide as adjuvant therapy for post TB arachnoiditis refractory to corticosteroids and anti-tubercular therapy (ATT). METHODS: This was a retrospective case series of patients of refractory post TB arachnoiditis leading to paraparesis and vision loss who received cyclophosphamide as an adjuvant therapy along with standard ATT and corticosteroids. These patients were treated with intravenous cyclophosphamide (dose 500 mg/m2) once a month for 4 consecutive months after informed written consent and were assessed clinically and radiologically before and after cyclophosphamide therapy. RESULTS: We had 4 patients with refractory post TB arachnoiditis of whom three became independently ambulatory. There was significant clinical as well as radiological improvement in all the patients. CONCLUSIONS: Cyclophosphamide therapy could be an effective therapy for patients with refractory post TB arachnoiditis. Well-designed randomized controlled studies are essential to study the safety and efficacy of cyclophosphamide in this condition.

A new surgical method for treating syringomyelia secondary to arachnoiditis following cervical spine surgery: the syringo-cisterna magna shunt.

PURPOSE: The 5-year postoperative failure rate of conventional shunt treatment for syringomyelia is 50%, with arachnoditis, shunt obstruction, and shunt malfunction being the most common causes. We report a new syringo-cisterna magna (SCM) shunt that allows syrinx cerebrospinal fluid (CSF) drainage normally into the cerebellomedullary cisterns through the subarachnoid space. METHODS: Between November 2012 and February 2017, six patients (mean age: 57.25 years; sex: four male and two female) received the SCM shunt. They had spinal cord injury, abscess formation after a spine operation, and cerebral meningitis-related syringomyelia (syrinx between C0 and T9), and presented sensory changes and motor weakness. Preoperatively and at 1 year postoperatively, the syrinx length and diameter were assessed using magnetic resonance imaging (MRI). Clinical outcomes were evaluated using the visual analog scale (VAS) and Japanese Orthopedic Association (JOA) scores. RESULTS: Motor weakness improved, pain subsided, and sensory disturbance resolved in all patients who returned to work within 6 weeks postoperatively. In all cases, the syrinx collapsed (length: 3.3 levels decreased; diameter: decreased from 7.90 to 4.64 mm, p = 0.046) on postoperative MRI. No patient experienced syrinx recurrence and shunt malfunction on MRI or showed spinal instability signs on plain radiography. The VAS (pre- vs post-shunt: 6.50 vs 3.83, p = 0.027) and JOA scores (pre- vs post-shunt: 10.00 vs 11.17, p = 0.167) were improved postoperatively. CONCLUSION: We developed a new shunting system allowing syrinx CSF drainage to the posterior fossa, with symptomatic improvement, minimal complications, and syrinx decrease on follow-up MRI. The SCM shunt is effective for treating syringomyelia.

Spinal arachnoiditis leading to recurrent reversible myelopathy: A case report.

Context: A patient followed in the outpatient spinal cord injury support clinic at a VA Medical Center with a prior remote history of a gunshot wound to the back and multiple prior myelograms presented with a recurrent waxing and waning weakness of the left lower extremity and intermittent incontinence of bowel and bladder.Findings: During the evaluation, the patient experienced an immediate albeit temporary improvement in symptoms after a diagnostic lumbar puncture performed for CT myelogram. The symptoms of myelopathy reoccurred several weeks, but then the patient had a similar experience with rapid improvement in symptoms after an accidental fall down a flight of steps. Subsequently, the foot weakness and incontinence returned one week later. The patient ultimately developed permanent improvement in signs and symptoms after surgical intervention which included intradural lysis of adhesions, incision of the arachnoid membrane and resection of a cystic lesion.Clinical relevance: Patients who experience unexpected, albeit transient improvement in myelopathic symptoms who are known or suspected to have arachnoiditis should be evaluated for surgically remediable lesions. Remediation of these lesions can potentially improve long term outcome.

MRI characteristics of syringomyelia associated with foramen magnum arachnoiditis: differentiation from Chiari malformation.

BACKGROUND: It is important to distinguish foramen magnum arachnoiditis (FMA) from Chiari malformation (CM) before surgery because the operative strategies for these diseases differ. In the current study, we compared pretreatment magnetic resonance imaging (MRI) of FMA with CM and investigated the MRI findings useful to differentiate between these diseases. METHODS: We retrospectively reviewed patients with FMA or CM aged ≥ 18 years who underwent surgeries at our institution between 2007 and 2019. The morphologies of the syrinx, neural elements, and posterior cranial fossa were preoperatively evaluated with MRI. We used the receiver operating characteristic (ROC) curve for the fourth ventricle-to-syrinx distance (FVSD). RESULTS: Ten patients with FMAs and 179 with CMs were included. FVSD in the FMA group was significantly shorter than that in the CM group (7.5 mm [IQR, 2.8-10 mm] in FMA vs. 29.9 mm [IQR, 16.3-52.9 mm] in CM, p < 0.0001). The other MRI findings that showed the height, size, and length of the syrinx; size of the foramen magnum; degree of cerebellar tonsillar descent; shape of the cerebellar tonsil; and dorsal subarachnoid space at the foramen magnum differed significantly between the two groups. The ROC curve analysis showed that patients whose FVSD was less than 11 mm could be diagnosed with FMA with a specificity of 90% and sensitivity of 96%. CONCLUSIONS: A more cranial syrinx development (FVSD < 11 mm) appears to be the characteristic MRI finding in FMA.

Arachnoiditis Ossificans of the Lumbar Spine: A Rare Cause of Progressive Cauda Equina Syndrome.

Arachnoiditis ossificans of the spine is a rare entity defined as an ossification of the leptomeninges resulting in neurologic decline. We describe the case of a 42-year-old woman, without any obvious predisposing factor, who presented with a progressive cauda equina syndrome. The imaging findings on magnetic resonance imaging were confusing by showing an atypical intraspinal lesion extending from L1 to S1. The computed tomography scan was more specific by showing suggestive images of a huge arachnoiditis ossificans of the lumbar spine. The patient underwent a large lumbar laminectomy with an incomplete resection of the ossified arachnoid. The histologic study confirmed the bony nature of the lesion. This illustrative case highlights the importance of helical computed tomography scan with multiplanar reconstruction for the diagnosis of arachnoiditis ossificans.

Spinal adhesive arachnoiditis: three case reports and review of literature.

Spinal adhesive arachnoiditis is a rare pathology involving pia mater of the spinal cord and nerve roots. It can potentially lead to disability-many patients end up wheelchair-bound due to subsequent paraparesis. It is an infrequent but possible cause of lower extremities weakness in patients with a history of spinal surgery, epidural anaesthesia, myelography or spinal tumors. Three patients, one male and two females, admitted to our unit due to paraparesis presented at least one of the above mentioned risk factors. Each of them had a severe course of illness-progressive paresis of lower extremities. All above cases were diagnosed with spinal adhesive arachnoiditis confirmed with Magnetic Resonance Imaging (MRI) scan-the most sensitive and specific diagnostic tool. Despite conservative treatment and intensive rehabilitation none of the presented patients preserved the ability to mobilise independently. Considering spinal adhesive arachnoiditis in patients with paraparesis and history of typical risk factors should be included in clinical diagnostic procedure.

Hemifacial Spasm Secondary to Arachnoiditis Due to Neurocysticercosis: Clinical Image.

Subarachnoid neurocysticercosis (NCC) is a form of NCC with cysticerci located in the subarachnoid space. This form of NCC can cause general and focal neurologic symptoms, and sometimes requires surgical intervention as a treatment. In this report, we present a rare case of hemifacial spasm secondary to arachnoiditis because of an NCC cyst in the cerebellopontine angle. The cysticercus was removed and the facial nerve was liberated via a retrosigmoidal approach. At 8-month follow-up, the patient reported no recurrence of symptoms. To our knowledge, this is the first case reported to surgically manage hemifacial spasm secondary to NCC arachnoiditis.

Compressive myelopathy secondary to posthemorragic arachnoiditis: Case report and literature review.

BACKGROUND: Spinal arachnoiditis is an arachnoid inflammatory process frequently caused by infection or spinal surgery; there are different degrees of severity, including arachnoid thickening and severe adhesive lesions that can lead to the development of arachnoid cysts. Non-traumatic subarachnoid haemorrhage (ntSAH) is a relatively uncommon cause of arachnoiditis; further complication with spinal cord compression (SCC) is even more unusual. METHOD: we describe a 70-year-old female, with SCC caused by arachnoid cysts. Her medical past history was relevant for an episode of ntSAH after rupture of a posterior communicating artery aneurysm, eight months prior to the onset of symptoms. We also present a literature review of previous published cases. RESULTS: we selected 23 articles with 24 case reports. A noticeable female predominance (11:1) was observed. It is more common between the fourth and fifth decades. The majority of cases (58 %) were secondary to aneurysmal SAH due to rupture of a posterior circulation aneurysm. The most common location of the cyst is in the cervicothoracic spine. The average time between the initial bleeding and symptom development is 3-6 months. The most frequently described treatment is laminectomy and marsupialization of the cyst, but reports show a high recurrence rate. CONCLUSIONS: ntSAH is an uncommon aetiology of arachnoiditis and arachnoid cysts. SCC from arachnoid cysts secondary to ntSAH is exceptional. Treatment through laminectomy has a relatively high recurrence rate (33 %). We present different hypotheses to try to explain how the alteration of cerebrospinal fluid (CSF) dynamics after ntSAH can lead to arachnoid cyst development and SCC. Although the small number of cases included in the present series precludes us to draw definite conclusions, ventriculoperitoneal shunt (VPS) placement can be considered as an alternative treatment in the management of known ntSAH patients that present recurrent symptomatic arachnoid cysts.

Minimally invasive asterional approach for microvascular decompression in trigeminal neuralgia.

OBJECTIVE: Trigeminal neuralgia (TN) is a neuropathic disorder that can be treated surgically. This study aimed to present the surgical findings and the clinical outcomes of 26 patients with TN treated by minimally invasive asterional surgery. METHODS: Longitudinal descriptive study. Twenty-six patients with TN underwent minimally invasive asterional surgery. The medical history, surgical findings, therapeutic response, and complications were registered. They were followed for 36 months. RESULTS: Nineteen cases were associated with vascular compression; five were associated with arachnoiditis. The two remaining cases were associated with multiple sclerosis and post-herpetic neuralgia. The pain was substantially reduced in all patients in the immediate postoperative period. At 36 months, in 25 patients, total or acceptable pain control was achieved. In the long term, 22 patients evolved with no permanent complications. CONCLUSION: The microvascular decompression surgery by an asterional approach is an alternative with similar results to the classic retrosigmoid approach to treat TN, but that adds the benefits of the principles of minimally invasive surgery. Constant efforts need to be made to optimize minimally invasive surgical techniques for TN.

Safety and Efficacy of Syringoperitoneal Shunting with a Programmable Shunt Valve for Syringomyelia Associated with Extensive Spinal Adhesive Arachnoiditis: Technical Note.

OBJECTIVE: Although syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be a progressive disease that has potentially devastating clinical consequences, its surgical resolution has remained poorly defined. The aim of the present study was to verify the safety and efficacy of syringoperitoneal shunting for syringomyelia associated with extensive SAA. METHODS: The present retrospective study included 15 patients who had undergone syringoperitoneal shunting with a programmable shunt valve for the diagnosis of syringomyelia associated with extensive SAA from October 2012 to June 2018. The shunt pressure was appropriately adjusted according to the postoperative sequential clinical condition and change in syringomyelia evaluated using magnetic resonance imaging. The average postoperative follow-up duration was 32.7 months. RESULTS: No surgery-related complications such as shunt dysfunction or infection occurred during the follow-up period, except for 2 patients with minor issues with the shunt tube. The average shunt pressure at the last follow-up examination was 4.5 cm H2O. The findings from the clinical assessment suggested that the average grade on the sensory pain scale was 2.9 before surgery and had improved significantly to 2.5 at the most recent follow-up examination. Radiological analysis suggested that improvement of syringomyelia was noted in 14 of the 15 patients (93.3%), with no cases of radiological aggravation. No recurrence of syringomyelia developed during the follow-up period in the present study. CONCLUSION: Syringoperitoneal shunting with a programmable shunt valve was safe and effective for clinical control of syringomyelia associated with extensive SAA. Long-term follow-up is mandatory to monitor for shunt dysfunction and mechanical trouble.

Arachnoiditis as an outcome factor for microvascular decompression in classical trigeminal neuralgia.

BACKGROUND: Neurovascular conflict is considered a key element of classical trigeminal neuralgia (TN) and consequently, microvascular decompression (MVD) is an effective treatment. Nevertheless, failures of MVD are described by many authors. In some patients, the arachnoid membranes surrounding the trigeminal nerve and neighbouring vessels may be thickened and adhesive. Here we analyse the impact of such focal arachnoiditis on outcome after MVD for TN. METHODS: A cohort of prospectively followed patients after their MVD was reviewed for intraoperative, imaging and clinical data if findings of arachnoiditis during MVD were described. Long-term outcome assessment was the main endpoint. RESULTS: We reviewed data from 395 MVD procedures, performed for TN from 2001 to 2014. Intraoperative evidence of focal arachnoiditis, as described by the surgeon, has been noted in 51 patients (13%). In 35 (68.6%), neuralgia was typical and in the other 17 (31.4%) it was atypical. As expected by definition, neurovascular conflict was found in 49 interventions (96%); it was predominantly arterial in 27 (52.9%). Accompanying arachnoiditis was encountered: mild in 20 interventions (39.2%), severe in 31 (60.8%). A successful result (BNI I or II) was achieved in 29 patients (56.9%). The other 22 patients (43.1%) had persistence or recurrence of pain. Overall KM probability of being pain free at 15 years was 72%. CONCLUSIONS: Intraoperative finding of arachnoiditis during MVD for classical trigeminal neuralgia is associated with poorer outcome than that of classical trigeminal neuralgia in general. This is particularly true for low grades of conflict.

A rare cause of progressive neuropathy: Arachnoiditis ossificans.

Arachnoiditis ossificans (AO) is a rare type of chronic arachnoiditis characterized by the presence of calcification or ossification of the spinal arachnoid which is usually associated with progressive neurological deficits. It is usually followed by prior history of trauma, surgery, infection, or myelography. Magnetic resonance imaging and computed tomography are the characteristics that are helpful in the diagnosis of most cases. Prognosis and treatment depends on the site and clinical presentation of the patients. We present a case of a young female who presented with a long-standing history of neurological symptoms and a intradural lesion mimicking a tumor.