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.

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.

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.

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 following spinal anesthesia-Case report and review of the literature]. / Arachnoiditis nach Spinalanästhesie ­ Fallbericht und Literaturübersicht.

A 61-year-old woman underwent a tension-free vaginal tape (TVT) operation due to stress incontinence. After technically difficult spinal anesthesia with two attempts the patient developed symptoms of nerve irritation, complained about neckache and headache and showed signs of agitation. The regimen was shifted to general anesthesia and surgery was performed. Because of postoperatively persistent headache and sensory disturbances an MRI scan of the lumbar spine was performed on the first postoperative day without pathological findings. The patient was able to leave the hospital after 1 week with significant relief of symptoms but 3 weeks later she developed neurocognitive impairment with memory deficits. A second MRI scan of the head now showed signs of disturbance of CSF circulation with hydrocephalus. Treatment was performed with drainage and ventriculoperitoneal shunt. Further evaluation showed a severe, multisegmental arachnoiditis and the patient developed a progressive paraparesis. The patient presented her case for assessment to a commission on medical malpractice 13 months after anesthesia. The commission detected no treatment errors. In connection to the case report a literature review of characteristics and etiologies of chronic adhesive arachnoiditis is given, which is a known but very rare complication of spinal anesthesia or similar procedures.

Spinal Syrinx Due to Lipiodol-Induced Arachnoiditis.

We present a case of a progressive symptomatic intramedullary cyst, diagnosed decades after Lipiodol injection. Lipiodol was originally administered intrathecally for the radiologic diagnosis of spinal masses. A link between the lesion and the history of Lipiodol injection was never suspected. Surgical exploration revealed a membrane above the lesion, separating the intradural space in a cranial and caudal compartment. On the level of the cyst, we identified glassy pearls containing a fatty liquid, compatible with Lipiodol deposits. We hypothesize that the syrinx is secondary to the impact of cerebrospinal fluid pulsations on the reactive membrane and that this membrane originated from an arachnoiditis caused by Lipiodol deposits. Lipiodol was indeed abandoned after it was found to cause arachnoiditis and neurologic sequelae. Despite the cessation of its usage, the causal role of Lipiodol in arachnoiditis and spinal cyst formation should still be considered, as symptoms may arise many years after Lipiodol administration.

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.

Spinal adhesive arachnoiditis mimicking sarcoid myelitis with nodular dural enhancement: A case report.

Spinal adhesive arachnoiditis (SAA) is a rare, but often devastating, cause of compressive myelopathy. We report a patient with SAA resulting in a longitudinally extensive T2-hyperintense spinal cord lesion with initial nodular pial and dural enhancement mimicking neurosarcoidosis. Neurologists should be aware of this entity, especially in patients who have pertinent risk factors, such as prior meningitis, spinal cord trauma, or surgery.

Recurrent arachnoid cysts secondary to spinal adhesive arachnoiditis successfully treated with a ventriculoperitoneal shunt.

Spinal adhesive arachnoiditis (SAA) with cyst formation secondary to infectious meningitis is a rare clinical entity. These cysts can compress the spinal cord and cause neurologic decline. We present a case of a patient who underwent resection for an intradural schwannoma which was complicated post-operatively by bacterial meningitis and development of several recurrent thoracic arachnoid cysts. After two separate thoracic decompressions with lysis of intradural adhesions, a permanent ventriculoperitoneal shunt (VPS) was eventually placed with complete recovery of his symptoms. Our review of the literature showed that CSF shunts have previously been successfully used to treat spinal fluid collections. Although there are many factors to consider when treating these patients, CSF diversion may be beneficial for similar SAA presentations in order to simultaneously treat and prevent recurrence of symptoms.

Case Report: A Case of Severe Cryptococcal Immune Reconstitution Inflammatory Syndrome Presenting with Brain and Intradural Abscesses in an HIV Patient.

Clinical worsening or new manifestation of cryptococcal disease following initiation of anti-retroviral therapy (ART) in an HIV patient is a hallmark of cryptococcal immune reconstitution inflammatory syndrome (C-IRIS). However, it can be difficult to distinguish IRIS from worsening or new infection. Here, we present a case of severe C-IRIS involving multiple cerebellar, spinal, and intradural abscesses and spinal arachnoiditis 7 months after ART initiation in an AIDS patient with uncertain prior ART compliance. He had multiple prior episodes of cryptococcal meningitis with complications necessitating ventriculoperitoneal shunt placement and was on suppressive fluconazole when he developed worsening brain manifestations. He received empiric anti-cryptococcal re-induction without improvement. All cerebrospinal fluid cultures remained sterile, with negative Cryptococcus PCR testing, and his condition continued to worsen prior to corticosteroid initiation. Ultimately, C-IRIS was diagnosed by brain biopsy. This case demonstrates an extreme in severity of C-IRIS and in the timeline of presentation after ART initiation.

Polyclonal lymphocytic infiltrate with arachnoiditis resulting from intrathecal stem cell transplantation.

Stem cell treatment outside of studied and approved medical indications can have unforeseen adverse consequences. Here, we present a 74-year-old male that underwent such therapy. The patient presented to our institution with progressive lower extremity weakness and urinary incontinence. He had previously undergone intrathecal stem cell therapy in Moscow, Russia for weakness and fatigue. Magnetic resonance imaging of his thoracic and lumbar spine showed marked enlargement of the cauda equina nerve roots and abnormal mass-like soft tissue involving the thoracolumbar thecal sac. Surgical biopsy of the intrathecal soft tissue showed polyclonal lymphocytic and glial cell proliferation. The patient's symptoms did not improve with medical treatment or radiation, and he is currently under observation after multidisciplinary evaluation. Our patient's experience illustrates one of the potential risks of "stem cell tourism" and exemplifies the imaging and histopathologic features of this rare entity. We also compare our patient's treatment with other similar examples of stem cell treatments in our institution and others. These have had a wide spectrum of results. In some instances, intrathecal stem cells have caused abnormal imaging findings without any associated patient symptoms. In extreme examples, however, stem cell treatments have resulted in central nervous system neoplasms. Our patient's lesion is quite unique, with only one similar lesion having been previously published.

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.

Endoscopic-Assisted Spinal Arachnoiditis Adhesiolysis and Placement of a Spinal Cysto-Subarachnoid Shunt.

BACKGROUND: Spinal arachnoiditis and associated arachnoid adhesions can cause debilitating neurological symptoms due to nerve root and spinal cord compression or tethering. Adhesiolysis using a microscopic approach has traditionally been used for this condition. This procedure has been further refined in recent years with the use of flexible endoscopes. CASE DESCRIPTION: We report the case of a patient with progressive thoracic myelopathy secondary to arachnoid adhesions associated with arachnoiditis. A minimally invasive technique of adhesiolysis and placement of a cysto-arachnoid shunt was performed with the assistance of a flexible endoscope. CONCLUSIONS: The present case report has highlighted the advantages of using a flexible endoscope to treat spinal arachnoiditis adhesiolysis and placement of a cysto-arachnoid shunt, including a smaller skin incision and extended visualization.

Arachnoiditis Ossificans: A Rare Etiology of Oil-Based Spinal Myelography and Review of the Literature.

BACKGROUND: Arachnoiditis ossificans (AO) is a rare entity characterized by the presence of calcified plaques formed by the metaplasia of arachnoid cells. Over 50 cases of AO have been reported, with predisposing factors including spinal trauma, hemorrhage, vascular abnormalities, and infection. The administration of oil-based contrast during myelography as an independent risk factor or in conjunction with other spinal pathology has been described in 9 cases. CASE DESCRIPTION: A 70-year-old woman presented for neurosurgical consultation in 2013 with a 2-year history of progressive midthoracic pain, right-sided chest wall allodynia, lower extremity weakness, and gait ataxia. Approximately 30 years ago, she received an oil-based contrast myelogram for investigation of spontaneous spinal hemorrhage. The procedure was well tolerated, and the patient experienced no allergic, hemorrhagic, traumatic, or infectious complications. No etiology was found for the spinal hemorrhage, and the patient recovered fully from that episode. Magnetic resonance imaging (MRI) of the thoracolumbar spine demonstrated multiple compressive intradural lesions in the upper thoracic spine and ventral tethering of the spinal cord at T7. MRI also demonstrated syringomyelia throughout the thoracic spine. Initially, the diagnosis of epidural mass or diastematomyelia was considered. To further characterize the epidural lesion, an unenhanced computed tomography (CT) scan was obtained, demonstrating a long segment of extensive calcification in the periphery of the thoracolumbar spine, with near-complete circumferential involvement from T5 to T11. The diagnosis of AO with extensive thoracic spine calcifications, syringomyelia, and spine cord tethering was made and confirmed at surgery. CONCLUSIONS: In addition to acute inflammation, oil-based contrast myelography also leads to arachnoiditis, calcification, and retained mass lesions because of its chronic inflammatory properties and slow resorptive rate. Three decades after its replacement with water-based contrast material, the chronic sequelae of oil-based contrast myelography may continue to manifest clinically and on CT imaging. Because of calcifications often encasing the spinal cord or nerve roots, management of AO is challenging, and neurologic deficits may persist even after surgery.

Adhesive arachnoiditis following inadvertent epidural injection of 2% chlorhexidine in 70% alcohol-partial recovery over the ensuing eight years.

We report a case of serious neurologic injury due to inadvertent epidural injection of 8 ml of the antiseptic 2% chlorhexidine in 70% alcohol during a procedure aimed to relieve the pain of labour. This resulted in immediate severe back pain, progressive tetraparesis and sphincter dysfunction caused by damage to the spinal cord and nerve roots. Subacute hydrocephalus necessitated drainage, but cranial nerve and cognitive function were spared. Magnetic resonance imaging documented marked abnormality of the spinal cord and surrounding leptomeninges. In the ensuing eight years, there has been clinical and electrophysiological evidence of partial recovery, but neurologic deficit remains severe.

Postarachnoiditis Anterior Spinal Arachnoid Cyst Formation with Compressive Myelopathy: Report of 2 Cases.

BACKGROUND: Spinal cystic arachnoiditis is a rare complication of a subarachnoid haemorrhage or infectious meningitis. The inflammatory process leads to fibrosis, adhesions, and in severe cases cyst formation. Large arachnoid cysts are an uncommon cause of compressive myelopathy. The majority are located posterior of the spinal cord at the thoracic level. Anterior cyst formation is exceptional, especially at the cervical region. CASE DESCRIPTION: We present 2 cases of progressive myelopathy secondary to anterior arachnoid cyst formation. In a 54-year-old female a large anterior symptomatic thoracic cyst arose 4 years after rupture of a posterior inferior cerebellar artery aneurysm. The other 59-year-old-patient, however, developed an anterior cervical cyst only weeks after a varicella meningoencephalitis. Both female patients were treated with a decompressive laminectomy and wide fenestration of the cysts. Partial recovery was obtained in 1 patient, but there was no improvement in the other case. CONCLUSIONS: Spinal cystic arachnoiditis with anterior cyst formation is an extremely rare complication of subarachnoid haemorrhage and infectious meningitis but can cause severe neurologic deficits. Clinicians should be aware of this rare complication. Due to the risk of irreversible spinal cord injury, rapid surgical intervention is recommended in most cases.