Delayed spinal arachnoiditis with syringomyelia following aneurysmal subarachnoid haemorrhage: a case report with patient experience.

BACKGROUND AND IMPORTANCE: Syringomyelia, or the formation of fluid-filled cysts within the spinal cord, associated with delayed spinal arachnoiditis is an uncommon complication of aneurysmal subarachnoid haemorrhage. To date, about 18 cases have been reported in medical literature, with just two reported in patients under the age of 35 years. CLINICAL PRESENTATION: A 27-year-old female patient complained of sudden, severe headaches in the occipital region, nuchal rigidity, and drowsiness when she presented at our institution. A head computed tomography scan revealed intraventricular bleeding in the lateral and fourth ventricles with more extensive haemorrhaging in the frontal horns. A left posterior inferior cerebellar artery (PICA) aneurysm was confirmed via digital subtraction angiogram, and endovascular embolization was done. Two years later, the patient reported intense pain in the lower back along with symptoms suggestive of spinal cord compression. Spinal magnetic resonance imaging (MRI) showed spinal adhesions from C1 to L4, syringomyelia with some vasogenic oedema extending from T3 to T9 level, and a cyst in the lumbar region. Consequently, a right hemilaminectomy was performed along with microsurgical release of arachnoid adhesions and placement of a subdural drain. Radiological and symptomatic improvements were observed. Since then, the patient's clinical condition has remained stable during the past three years of follow-up visits. CONCLUSIONS: Literature on optimal treatment modalities and patient prognosis is scarce and debated. The time for symptom improvement depends on the level and extent of spinal cord involvement. Rehabilitation may be required for most patients, as complete symptomatic recovery may not be attainable.

Craniovertebral and spinal adhesive arachnoiditis: a late complication of ruptured vertebral and posterior inferior cerebellar arteries aneurysms.

BACKGROUND: Adhesive arachnoiditis is a rare yet serious complication that may occur following subarachnoid hemorrhage (SAH). In this circumstance, it is mainly due to ruptured vertebral artery (VA) or posterior inferior artery (PICA) aneurysms. It disrupts cerebrospinal fluid (CSF) flow leading to complications such as spinal arachnoiditis, syringomyelia, trapped 4th ventricle, or a combination of these conditions. Evidence for effective treatment strategies is currently limited. We aimed to review the epidemiology, clinical characteristics, treatment, complications, outcomes, and prognosis of cranio-vertebral junction and spinal adhesive arachnoiditis resulting from ruptured VA and PICA aneurysms. METHODS: This study involved a comprehensive literature review and complemented by our own case. We focused on adult cases of arachnoiditis, syringomyelia, and trapped 4th ventricle with SAH caused by ruptured VA or PICA aneurysms, excluding cases unrelated to these aneurysms and those with insufficient data. RESULTS: The study included 22 patients, with a mean age of 52.4 years. Symptoms commonly manifest within the first year after SAH and timely diagnosis requires a high index of suspicion. Treatment approaches included lysis of adhesions and various shunt procedures. Most patients showed improvement post-treatment, though symptom recurrence is significant. CONCLUSION: Adhesive arachnoiditis is a critical complication following SAH, most commonly from ruptured VA and PICA aneurysms. Early detection and individualized treatment based on the type of arachnoiditis and CSF flow impact are crucial for effective management. This study underscores the need for tailored treatment strategies and further research in this field.

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.

Giant intradural extramedullary spinal ependymoma, a rare arachnoiditis-mimicking condition: case report and literature review.

BACKGROUND AND IMPORTANCE: Ependymomas are tumours arising from the ependymal cells lining the ventricles and the central canal of the spinal cord. They represent the most common intramedullary spinal cord tumour in adults and are very rarely encountered in an extramedullary location. Only 40 cases of intradural extramedullary (IDEM) ependymomas have been reported, all of which were diagnosed pre-operatively as IDEM ependymomas on contrast-enhanced MRI. CLINICAL PRESENTATION: We report a 23-year old male presenting with rapidly worsening signs and symptoms of spinal cord disease. A spinal MRI demonstrated a posterior multi-cystic dilatation extended between T1 and T12. Post-contrast sequences showed peri-medullar leptomeningeal enhancement and the diagnosis of spinal arachnoiditis was made. The patient underwent surgery and the spinal cord appeared circumferentially wrapped by an irregular soft tissue. The tissue was sub-totally removed and the pathological diagnosis was ependymoma WHO grade II. The patient experienced an excellent neurological recovery and no further treatments were administered. A small residue is now stable at 2.5 years follow-up. CONCLUSIONS: Giant IDEM ependymomas are rare entities and pre-operative diagnosis can be challenging in some cases. Surgery represents the main treatment option being resolutive in most cases.

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.

Reasons for re-hospitalization in patients with tuberculous meningitis, and its impact on outcome: a prospective observational study.

BACKGROUND: Patients with tuberculous meningitis may worsen despite being treated adequately with anti-tuberculosis drugs. This worsening may lead to re-hospitalization. The exact frequency and causes of re-hospitalization have not been studied previously. We aimed to study the causes of clinical worsening leading to re-hospitalization and its impact on prognosis. METHODS: This was a prospective observational study. Newly diagnosed patients with tuberculous meningitis (N = 150) were enrolled. Baseline clinical evaluation, neuroimaging, and cerebrospinal fluid examination were performed. Anti-tuberculosis drug regimen and corticosteroids were given as per WHO guidelines. Patients were followed for 6 months. Re-hospitalized patients were worked up and clinical evaluation, neuroimaging, and cerebrospinal fluid examination were performed again. Outcome assessment was done at the end of 6 months, and a modified Barthel index of ≤ 12 was considered a poor outcome. RESULTS: Twenty-three (15.3%) out of 150 patients needed re-hospitalization. The median time between discharge after the first hospitalization and re-hospitalization was 60 days. The common reasons for re-hospitalization were paradoxical neurological deterioration seen in 19 (82.6%) out of 23 patients, followed by drug toxicities (N = 2) and systemic involvement (N = 2). Paradoxically developed spinal arachnoiditis and opto-chiasmatic arachnoiditis were amongst the predominant reasons for re-hospitalization. At six months, re-hospitalization was an independent predictor of poor outcome (OR = 7.39, 95% CI 2.26-24.19). CONCLUSION: Approximately 15% of tuberculous meningitis patients needed re-hospitalization. Paradoxically developed spinal arachnoiditis and opto-chiasmatic arachnoiditis were predominant reasons for re-hospitalization. Re-hospitalization adversely affected the outcome.

Comparison of dexamethasone regimens in tubercular meningitis (TBM): a randomized open label clinical trial.

INTRODUCTION: Corticosteroids are used as adjunctive treatment in tuberculous meningitis (TBM). However, there is no universally accepted regimen, type, duration, or route of steroid administration. METHODOLOGY: In a randomized open labelled pilot study, TBM patients were divided into overlap oral dexamethasone (OOD) and direct oral dexamethasone (DOD) arms. The total duration of steroid administration was 8 weeks. The primary outcome was symptomatic resolution at 1 month post randomization. The secondary outcomes were mortality and modified Rankin scale (mRS) at 3 and 6 months after initiation of steroids. RESULTS: Symptomatic resolution after one month of randomization in 53 randomized patients was similar in OOD (71.4% (15/21)) versus DOD ((85.0% (17/20)) arm (p value:0.45). Median mRS was also similar in OOD versus DOD (OOD: 2.5 (IQR: 1.0; 6.0) versus DOD: 1.0 (IQR: (0.0; 4.0); p value: 0.31)) arm at 6 months. The mortality at 6 months was 31.8% (7/22) in the OOD versus 20.0% (4/20) in the DOD arm (p value: 0.49). CONCLUSIONS: In this open label pilot study, the outcomes were similar in OOD versus DOD arms in terms of symptomatic resolution at 1 month, and morbidity, and mortality at 3 and 6 months. Patients with stage I to III TBM may be given injectable steroids for 1 week after which they may be switched to oral steroid. This regime cannot be applied to stage IV TBM and patients with complications like optico-chiasmatic or spinal arachnoiditis or vasculitic infarcts.

The leptomeninges as a critical organ for normal CNS development and function: First patient and public involved systematic review of arachnoiditis (chronic meningitis).

BACKGROUND & IMPORTANCE: This patient and public-involved systematic review originally focused on arachnoiditis, a supposedly rare "iatrogenic chronic meningitis" causing permanent neurologic damage and intractable pain. We sought to prove disease existence, causation, symptoms, and inform future directions. After 63 terms for the same pathology were found, the study was renamed Diseases of the Leptomeninges (DLMs). We present results that nullify traditional clinical thinking about DLMs, answer study questions, and create a unified path forward. METHODS: The prospective PRISMA protocol is published at Arcsology.org. We used four platforms, 10 sources, extraction software, and critical review with ≥2 researchers at each phase. All human sources to 12/6/2020 were eligible for qualitative synthesis utilizing R. Weekly updates since cutoff strengthen conclusions. RESULTS: Included were 887/14286 sources containing 12721 DLMs patients. Pathology involves the subarachnoid space (SAS) and pia. DLMs occurred in all countries as a contributor to the top 10 causes of disability-adjusted life years lost, with communicable diseases (CDs) predominating. In the USA, the ratio of CDs to iatrogenic causes is 2.4:1, contradicting arachnoiditis literature. Spinal fusion surgery comprised 54.7% of the iatrogenic category, with rhBMP-2 resulting in 2.4x more DLMs than no use (p<0.0001). Spinal injections and neuraxial anesthesia procedures cause 1.1%, and 0.2% permanent DLMs, respectively. Syringomyelia, hydrocephalus, and arachnoid cysts are complications caused by blocked CSF flow. CNS neuron death occurs due to insufficient arterial supply from compromised vasculature and nerves traversing the SAS. Contrast MRI is currently the diagnostic test of choice. Lack of radiologist recognition is problematic. DISCUSSION & CONCLUSION: DLMs are common. The LM clinically functions as an organ with critical CNS-sustaining roles involving the SAS-pia structure, enclosed cells, lymphatics, and biologic pathways. Cases involve all specialties. Causes are numerous, symptoms predictable, and outcomes dependent on time to treatment and extent of residual SAS damage. An international disease classification and possible treatment trials are proposed.

"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.

Magendie's foramen debridement and catheterisation for the treatment of syringomyelia due to diffuse craniocervical junction arachnoiditis. A case report and technical note.

A 36 year old woman was referred to our department for symptomatic lumbar spinal arachnoiditis following an epidural anaesthesia for childbirth. She did not had other known causative factor and she was free of any neurological symptoms before. She rapidly developed lower limbs impairment by compressing intradural lumbar collections and arachnoiditis requiring surgical decompression and subsequently internal cerebrospinal fluid shunting for acute hydrocephalus. Three years and the half later, she developed a severe tetraparesis due to a massive syrinx consecutive to the fourth ventricle outlets obstruction cause by the ongoing diffuse craniocervical junction arachnoiditis. Our aim was to treat all the problems in one step. An open fourth ventriculostomy of the Magendie's foramen with catheter insertion from the fourth ventricle down to the upper cervical subarachnoid space improve both the patient status and imagery.

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.