A rare case of bilateral en plaque meningioma presenting as a bilateral exophthalmos. A case report.

En plaque meningioma is a rare type of meningioma characterized by an infiltrative nature, sheet-like growth, and at times invading the bone. We report here a case of en plaque meningioma. The patient was a 66-year-old woman presenting with headache and painful bilateral proptosis. Cerebral magnetic resonance imaging revealed a bilateral en plaque meningioma showed as a bilateral hyperostotic of greater wing sphenoid bone associated with bilateral thickening and enhancement of the dura in the anterior temporal area and the retroclival region invading bilaterally the cavernous sinus, the prechiasmatic portion of the optic nerve, and the lateral rectus muscle of the orbit through the superior orbital fissure causing bilateral exophthalmia. Due to invasion of the cavernous sinus and the orbital apex, a subtotal but extensive removal combined with bony decompression of the cranial nerves at the superior orbital fissure and optic canal followed by adjuvant radiotherapy frequently produces good functional and cosmetic results, and over a 3-year period, follow-up magnetic resonance imaging scans showed no obvious signs of recurrence.

An Incidental Finding of Idiopathic Hypertrophic Pachymeningitis: A Case Report.

In this case report, we discuss and explore the clinical, laboratory, and imaging findings, as well as the treatment options and follow-up measures, in an 83-year-old patient with idiopathic hypertrophic pachymeningitis (IHP), a rare disorder characterized by fibrosing, hypertrophic inflammation that thickens the dura mater. An 83-year-old female with a medical history of hypertension and hyperlipidemia presented with speech arrest and was taken to the emergency department, where she received a stroke code, a CT scan, and an MRI. The MRI results showed a temporal lobe meningioma and a pan-cranial pachymeningitis encasing the entire brain and cerebellum and extending into the upper cervical spine. Multiple unsuccessful attempts at a lumbar puncture were made, so a dural biopsy specimen was obtained, which revealed no malignant process. A cerebral spinal fluid specimen (CSF) from the biopsy showed minimal white blood cells (WBCs) which ruled out infection. Idiopathic hypertrophic pachymeningitis was the given diagnosis based on the apparent MRI findings. The patient was treated in the hospital for four days with IV methylprednisolone and discharged on oral methylprednisolone for four to six weeks.

Insights into neurosarcoidosis: an imaging perspective.

Neurosarcoidosis is a complex and multifaceted inflammatory disorder affecting the nervous system. The disease, characterized by non-caseating granulomas, primarily involves the central and peripheral nervous systems. Neuro-logical manifestations vary widely and can include cranial nerve palsies, meningeal involvement, parenchymal lesions, hydrocephalus, and more. Diagnosis remains challenging due to the lack of specific imaging features, necessitating a combination of clinical evaluation, cerebrospinal fluid analysis, imaging studies, and sometimes tissue biopsy. This review article underscores the importance of the identification of various imaging features to mitigate the mortality and morbidity associated with neurosarcoidosis.

Rapid bilateral visual loss as the initial clinical manifestation in idiopathic hypertrophic cranial pachymeningitis.

A 59-year-old patient presented with 4-day acute painless bilateral visual loss, MRI results showed dura enhancement of the frontal, anterior cranial fossa. The patient was considered to have idiopathic hypertrophic cranial pachymeningitis based on laboratory tests and MRI data. After treatment with hormones, the visual acuity obviously improved.

Neurosurgical and neuro-immunological management of IgG4-related hypertrophic sclerosing pachymeningitis. A literature survey and discussion of a unique index case.

BACKGROUND: Dural thickening is observed in lymphoma, dural carcinomatosis, meningioma, tuberculosis, and autoimmune diseases. We encountered a patient with dural thickening and complaints of neck and back pain, numbness and loss of strength in the hands. The patient also suffered from polychondritis and had previously received steroid and methotrexate treatment for this indication. The patients' serum was also positive for ANA, yet she did not have any other findings suggesting lupus. Our radiological and pathological analysis revealed IHSP (IgG4-related hypertrophic sclerosing pachymeningitis). In this review study, we provided a detailed literature survey to increase the awareness about IHSP in the neurosurgical community. METHODS: MRI (magnetic resonance imaging)-based radiological analyses revealed a posterior extramedullary spinal mass extending from C2 to T2-T3 level. The dural mass was surgically excised and a broad panel of immunohistochemical markers including S100, EMA, CD246/ALK-1, CD45, CD20, CD79a, CD138, CD68, CD1a and CD34 was studied. Immunoglobulin heavy chain/kappa chain gene rearrangement analysis was performed which ruled out a lymphoproliferative disorder. RESULTS: MRI and pathological findings suggested IHSP. As the disease relapsed with a new anterior extramedullary multilobulated lesion extending from C5 to T1 level, the patient is now closely monitored for further medical and surgical treatment. CONCLUSIONS: IHSP is a relatively novel entity of hypertrophic pachymeningitis and should be included in the differential diagnosis of dural thickening. The fibrosis accompanying IHSP may not respond to medical treatment, which includes steroids and immunosuppressive agents. Additionally, neurological deficits, seizures, spinal decompression, hydrocephalus, or brainstem compression necessitate early surgical intervention. A continued vigilance is also necessary as the disease may relapse long-term following surgical treatment.

Idiopathic Hypertrophic Pachymeningitis: Does Earlier Treatment Improve Outcome?

Background/goal: Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. This condition has largely been reported in adults, but there are very few reports in children. METHODS: We describe a 14-year-old boy with idiopathic hypertrophic pachymeningitis, who presented with deteriorating vision on a background of severe headache. We evaluated pediatric cases of hypertrophic pachymeningitis and compared treatments and their relation to outcomes. RESULTS: There are only eleven pediatric cases of hypertrophic pachymeningitis reported in the literature. In the patients treated with steroids either at presentation or subsequent relapses, a good response was reported. In the cases with delayed initiation of steroid treatment, this was often related to an incomplete recovery. In our patient, this delay may have contributed to his poor visual outcome. CONCLUSIONS: Early initiation of steroid treatment in children with idiopathic hypertrophic pachymeningitis may improve outcomes.

Importance of actively suspecting intracranial hypotension with diagnostic imaging prior to lumbar puncture.

It is important to suspect intracranial hypotension based on distinctly frequent orthostatic headaches and diffuse dural hyperplasia. Lumbar puncture is a procedure prone to complications, especially in patients with already existing intracranial hypotension.

Idiopathic Hypertrophic Spinal Pachymeningitis: A Diagnostic Challenge: A Case Report and Review of the Literature.

Idiopathic hypertrophic pachymeningitis (IHP) can resemble other disorders associated with spinal compression. It is a rare inflammatory fibrosing disease of the dura of unidentified etiology and is considered a diagnosis of exclusion. We present a case of idiopathic hypertrophic spinal pachymeningitis occupying a long segment of cervical dura. This is a case of 38-year-old female patient, who suffered progressive neck pain for 2-year duration. Examination revealed spasticity in all four limbs, plus three symmetric reflexes all over, and the sensory level at T4. Magnetic resonance imaging showed spinal cord compression by a thickened anterior and posterior dura adjacent to the cord from C2 to C7. The diagnosis of spinal IHP was confirmed through biopsy. The patient improved after treatment with corticosteroids. Early surgical intervention with postoperative corticosteroid therapy is a known treatment for this disease, as a way to prevent irreversible neurological damage.

Neurosyphilis: The shape of a rising threat.

This report describes a case of neurosyphilis presenting with memory disturbances, attention deficit, and acute psychotic decompensation in an immunocompetent man. Despite the known connection of neurosyphilis with psychiatric symptoms, this cause often remains unrecognized. This report emphasizes the importance of maintaining a suspicion for the disease in patients with vague symptoms and describes the diagnostic difficulties.

Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case.

Background Hemicrania continua (HC) is a primary headache syndrome characterized by a unilateral, moderate, continuous headache with exacerbations marked by migrainous and cranial autonomic symptoms. However, clinical phenotypes similar to primary HC may be subtended by several disorders. Case report We report the case of a 62-year-old man experiencing, over the previous year, a headache completely consistent with HC and its absolute responsiveness to indomethacin therapy. Later, the patient developed diplopia caused by sixth cranial nerve palsy ipsilateral to headache. In this frame, clinical, laboratory and neuroimaging characteristics supported the diagnosis of idiopathic hypertrophic pachymeningitis (IHP). Conclusions IHP is a rare fibrosing inflammatory disorder leading to a localized or diffuse dura mater thickening. IHP clinical manifestations are a progressively worsening headache and signs related to cranial nerves involvement and venous sinus thrombosis. Here, we report, for the first time, a HC phenotype subtended by IHP.

Ipsilateral Dural Thickening and Enhancement: A Sign of Isolated Cortical Vein Thrombosis? A Case Report and Review of the Literature.

BACKGROUND: Isolated cortical vein thrombosis (ICVT) being a rare condition (6% of intracranial vein thromboses), no clinical guidelines and few radiologic clues to it have been established. ICVT mostly appears in conjunction with sinus vein thromboses. ICVTs primarily occur during pregnancy and puerperium (35%). The great variability of cortical veins and difficulty identifying small occluded vessels complicate ICVT diagnosis. We present the first case of isolated ipsilateral dural thickening and enhancement as a potential radiologic sign of ICVT shown on magnetic resonance imaging. CASE DESCRIPTION: A 30-year-old woman presented with sudden position-independent severe headache and neck pain 2 weeks postpartum. Standard magnetic resonance imaging revealed ipsilateral dural thickening and enhancement of the meninges (left hemisphere). The symptoms and findings were interpreted as a post-lumbar puncture syndrome associated with the epidural anesthesia during labor and birth. Deteriorating, the patient was referred to our hospital after a computed tomography scan had revealed atypical left parietal intracranial hemorrhage. Digital cerebral subtraction angiography confirmed a left parietal ICVT as the underlying disease. When systemic anticoagulation was initiated, the patient's condition further deteriorated. Progressive aphasia and right-sided face and arm weakness and numbness developed as a result of increased intracranial hemorrhage volume. We therefore performed craniotomy and hematoma evacuation, after which the patient fully recovered. CONCLUSIONS: We hypothesize that ipsilateral dural thickening and enhancement in patients presenting with severe headache may indicate ICVT and we suggest further diagnostic workup, using venographic study such as computed tomographic venography or magnetic resonance venography. In an inconclusive venographic study with high clinical suspicion for ICVT, catheter cerebral angiography is indicated.

Bilateral Vision Loss Secondary to Pachymeningitis in a Patient with IgG4-Related Disease.

IgG4-related disease (IgG4-RD) is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges. A proportion of idiopathic hypertrophic pachymeningitis cases may involve a component of meningeal IgG4-RD. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

Idiopathic hypertrophic spinal pachymeningitis : report of two cases and review of the literature.

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.

Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.