Neurosarcoidosis.

Sarcoidosis affects the nervous system in 5% of cases. 60% of cases involve the cranial and peripheral nerves, the remainder the central nervous system, in which a leptomeningitis, a pachymeningitis and a vasculitis may arise. Stroke and cerebral haemorrhage may occur, and certain infections in the brain are more likely with sarcoidosis.

Gastrointestinal dysmotility complicating Behçet's syndrome: description of a newly recognised clinical phenotype.

Objectives: Gastrointestinal dysmotility may arise in a variety of auto-immune and auto-inflammatory diseases and hitherto has not been described in Behçet's syndrome (BS).

Methods: We present data on a cohort of seven patients under our care who presented with symptoms of and investigations compatible with an immune associated disorder of gastrointestinal motility, or enteric neuropathy.

Results: We describe the clinical features and investigation results.

Neurological involvement by Behçet's syndrome: clinical features, diagnosis, treatment and outcome.

Neurological involvement in Behçet's syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur.

Management of neurosarcoidosis.

This is a brief and purposefully practical approach to the therapeutic and rehabilitative management of patients affected by neurological complications of systemic sarcoidosis. The review notes the drugs used and their monitoring, and their role in the series of clinical subgroups identified to form the condition. Treatment guidelines for individual clinical subtypes of the disorder are provided, and the importance of rehabilitative measures and lifestyle changes are emphasised.

Idiopathic intracranial hypertension presenting as iron deficiency anemia: a case report.

Background: The presentation of idiopathic intracranial hypertension (IIH) in association with iron deficiency anemia (IDA) is rare.

Case Presentation: This case report depicts the unusual case of a 31-year-old woman of mixed Jamaican and English heritage with IIH who presented initially as IDA in the context of menorrhagia. Subsequent ophthalmic review, lumbar puncture, cerebrospinal fluid analysis and neuroimaging studies revealed severe bilateral optic disc swelling and raised intracranial pressure in keeping with IIH.

Neurosarcoidosis: clinical manifestations, investigation and treatment.

Sarcoidosis affects the nervous system in 10% of cases. When it does so it can affect any part of the nervous system and with all degrees of severity. It forms part of the differential diagnosis in inflammatory, infective, neoplastic and degenerative neurological diseases and may be very difficult to diagnose without histological confirmation.

Sarcoidosis of the central nervous system: Safety and efficacy of treatment, and experience of biological therapies.

Objectives: Neurological complications of sarcoidosis are uncommon and the natural history and optimal treatments under-researched. With the advent of biological therapies, it is important to define the clinical characteristics and immunopathology of the disease.

Patients And Methods: Patients referred to and treated within the Centre for Neurosarcoidosis over a 15 year period who had biopsy proven "highly probable" disease of the central nervous system were studied prospectively.

Granulomatous CNS inflammation associated with seminoma.

Two cases in which a neurological disorder was identified pathologically to be due to a granulomatous infiltration were found after diagnosis to have an associated testicular seminoma with pathologically proven lymphatic metastasis. We present the clinical and imaging features, and pathological appearances of the lymphatic tissue and the brain. We summarise the literature to date and discuss the pathogenesis of the disorder and its treatment.

Sarcoidosis of the central nervous system: clinical features, imaging, and CSF results.

Objective: Neurological complications of systemic sarcoidosis are uncommon and the natural history and optimal treatments under-researched. With the advent of modern biological therapies, it is important to define the clinical characteristics and immunopathology of the disease.

Methods: Patients referred to and treated within the Centre for Neurosarcoidosis over a 15 year period who had biopsy-proven "highly probable" disease of the central nervous system were studied prospectively.

Optic neuropathy associated with systemic sarcoidosis.

Objective: To identify and follow a series of 52 patients with optic neuropathy related to sarcoidosis.

Methods: Prospective observational cohort study.

Results: The disorder was more common in women and affected a wide age range.

Optic neuropathy in Behçet's syndrome.

This paper presents the clinical features, investigation results and response to treatment of four cases of primary optic neuropathy in Behçet's syndrome, with a comprehensive literature search for all other published cases. A total of 20 cases has been identified; the clinical syndrome is a unilateral or bilateral optic neuropathy of inflammatory type in which pain arises in 20 % and optic disc swelling seen in 45 % of cases. The condition was relapsing in four cases, and a progressive optic neuropathy was seen in one case.

Neuro-Behçet's disease: epidemiology, clinical characteristics, and management.

Behçet's disease (BD) is a multisystem relapsing inflammatory disorder of unknown cause. In neuro-BD (NBD), the CNS can be involved in one or both of two ways: first, and most commonly, through the development of an immune-mediated meningoencephalitis, which predominantly involves the brainstem, but can also involve the basal ganglia, thalamus, cortex and white matter, spinal cord, or cranial nerves; and second, as a consequence of thrombosis within the dural venous sinuses. Headache is a common symptom in BD and does not necessarily indicate CNS involvement.

Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement.

Rosai-Dorfman disease is associated with a histiocytic infiltration of lymphoid tissue, but may also involve the orbit, nasopharynx, respiratory pathways, gastrointestinal tract, endocrine glands (particularly the thyroid), bone, and skin. Neurologic manifestations are rare, occurring in 4% of one series, and the diagnosis is often not made until pathology is available. Using data from two cases, we present the clinical manifestations, pathology, and treatment, and review the literature regarding the ocular and neurologic manifestations.