Experience of using Rituximab in neurological practice (literature review and own observation)

Currently, due to the lack of specific etiotropic therapy, rituximab is widely used for the treatment of most autoimmune diseases of the central and peripheral nervous system. Rituximab is a chimeric monoclonal antibody with specificity for CD20, the antigen found on the surface of normal and malignant B-lymphocytes. It is used mainly in hematological practice. It is used off-label for the treatment of neurological diseases. The world literature describes the use of rituximab for the treatment of such pathologies as autoimmune encephalitis, neuromyelitis optica spectrum disorder, multiple sclerosis, primary angiitis of the central nervous system, immune-mediated inflammatory polyneuropathy, myasthenia gravis, refractory to basic immunosuppressive therapy. This article provides an overview of the world literature on the use of rituximab in neurological practice, describes our own experience of its use on the basis of the Department of Neurology № 1 of Pavlov University (Saint Petersburg, Russia).

Headache and Hemiparesis in Middle Age

A 53-year-old man with hypertension, hyperlipidemia, and a remote, cryptogenic, multifocal, posterior circulation ischemic stroke, came to the emergency department with 1 day of vertigo, ataxic gait, nausea, occipital headache, and painless binocular diplopia. Symptoms were present upon awakening on the day of presentation and progressed throughout the day. The erythrocyte sedimentation rate was 10 mm/h and C-reactive protein level was less than 3 mg/L. Cerebrospinal fluid examination indicated mild lymphocytic pleocytosis with 9 cells/µL, protein value of 45 mg/dL, and glucose level within normal limits. Brain magnetic resonance imaging and magnetic resonance angiography with contrast demonstrated a left caudate head infarction and leptomeningeal and perivascular enhancement involving bilateral temporal lobes, basal ganglia, and frontal lobes. Right middle cerebral artery wall enhancement was also noted. Conventional cerebral angiography showed diffuse dilatation and mural irregularity of the right middle cerebral artery M1 segment, as well as dilatation of the first 2 mm of the left anterior cerebral artery A1 segment. These findings were associated with vessel wall gadolinium enhancement on magnetic resonance imaging, which raised concern for vasculitis. The patient was diagnosed with primary angiitis of the central nervous system. The diagnosis was based on the presence of multiple ischemic infarcts, without cardioembolic source, abnormal brain magnetic resonance imaging and magnetic resonance angiography findings consistent with vasculitis, and the absence of systemic vasculitis, infection, and cancer. After diagnosis, the patient was started on intravenous methylprednisolone, followed by oral prednisone and intravenous cyclophosphamide. Because of some new areas of enhancement on magnetic resonance imaging, the patient was subsequently treated with rituximab. Clinical and radiologic remission was achieved, although the patient had permanent residual gait difficulties. Central nervous system vasculitis is an inflammatory process of the blood vessels in the brain, meninges, and spinal cord. It is called primary angiitis of the central nervous system when the process is limited to the brain and, rarely, the spinal cord. In other circumstances, central nervous system vasculitis can be secondary to a systemic inflammatory syndrome or infectious process.

Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey

Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS. Due to its rarity, variable clinical representation, and the lack of a diagnostic criteria and therapeutic plans, diagnosis and treatment of cPACNS is challenging and approaches vary. This survey collected information on diagnostic and therapeutic approaches to sv-PACNS. It was shared with international clinician networks, including the German Society for Paediatric Rheumatology, the Paediatric Rheumatology European Society, the “Network Paediatric Stroke,” and members of the American College of Rheumatology/CARRA Paediatric Rheumatology list server. This project has shown consensus in numerous diagnostic and therapeutic treatment approaches, highlighting key areas which will be utilised to develop statements in the use of expert consensus meetings to standardise diagnostic and therapeutic approaches in this rare inflammatory disease.

Primary phlebitis of central nervous system revealed by black-blood magnetic resonance imaging

Primary phlebitis of the central nervous system (PPCNS) is a rare condition that might be a subset of primary angiitis of the CNS. In this case report, the patient was a 39-year-old man with a 2-week history of anterograde amnesia and abnormal behaviours. Black-blood MRI (BB-MRI) showed contrast enhancement of the left basilar vein and cerebral superficial veins. Angiography showed unremarkable change in arteries. After a thorough differential diagnosis, we diagnosed PPCNS and then administered methylprednisolone pulse and cyclophosphamide pulse. The neuropsychological symptoms and MRI findings gradually improved, and after 2 months, the dose of prednisolone was gradually reduced to 20 mg. No recurrence was observed. This case shows that BB-MRI may be useful for diagnosing PPCNS.

Elevated intracranial pressure requiring decompressive craniectomy in a child with progressive primary angiitis of the central nervous system: a case report

Background Elevated intracranial pressure is a potentially catastrophic complication of neurologic injury in children. Successful management of elevated intracranial pressure requires prompt recognition and therapy directed at both reducing intracranial pressure and reversing its underlying cause. A rare condition that causes elevated intracranial pressure is childhood primary angiitis of the central nervous system, which is a rare inflammatory central nervous system disease that poses diagnostic and therapeutic challenges. To our knowledge, this is the first reported case of angiography-positive progressive childhood primary angiitis of the central nervous system requiring decompressive hemicraniectomy for refractory elevated intracranial pressure in children. Case presentation We report the case of a 5-year-old Saudi girl who presented to the pediatric emergency department with fever and new-onset status epilepticus. She had elevated inflammatory markers with radiological and histopathological evidence of angiography-positive progressive childhood primary angiitis of the central nervous system, complicated by elevated intracranial pressure. Despite medical management for both childhood primary angiitis of the central nervous system and elevated intracranial pressure, her neurological status continued to deteriorate and the elevated intracranial pressure became refractory. She developed right uncal, right subfalcine, and tonsillar herniation requiring decompressive hemicraniectomy with a favorable neurological outcome. Conclusion Decompressive craniectomy might be considered in cases of angiography-positive progressive childhood primary angiitis of the central nervous system with elevated intracranial pressure refractory to medication. A multidisciplinary approach for the decision of decompressive craniectomy is advised to ensure patient safety and avoid possible morbidities and mortality.