Introduction
Purpose of review
Definition and epidemiology
Background
Natural history
Diagnosis
When should neurosarcoidosis be suspected?
Clinical presentation | Characteristics in NS |
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Mostly this is asymptomatic and inferred from CSF abnormalities. Where patients are symptomatic, the presentation is usually subacute or chronic | |
Typical CSF finds are: pleocytosis (<220 cells/mm3) with a lymphocytic predominance and/or raised protein (<4.3 g/l). Reduced CSF glucose is also reported | |
This may be of acute or subacute onset. CSF and imaging abnormalities usually confirm a neuro-inflammatory basis | |
This is the most frequently reported manifestation of NS. Any cranial nerve can be involved but facial and optic nerves are most frequently affected | |
Facial nerve palsies often spontaneously remit and carry a good prognosis | |
Cranial oligoneuropathy or polyneuropathy (e.g. bilateral facial nerve palsy) is suggestive of NS | |
Optic nerve involvement may have a more difficult disease course with refractory disease and relapse on corticosteroid dose reduction | |
A pharynx, soft palate and vocal cord syndrome from glossopharyngeal and vagus nerve involvement is recognised | |
Basal meningitis may be the pathophysiological substrate of cranial neuropathies | |
Lesions may be multiple and often enhance. Biopsy of mass lesions is recommended for a definitive diagnosis | |
Behaviour change, confusional states and psychosis are reported | |
Usually of insidious onset, due to suprasellar inflammatory lesions. The most eminent symptoms are bitemporal visual failure, polydipsia and polyuria (diabetes insipidus), and galactorrhoea | |
Symptoms may arise from hypothalamic dysfunction, hypopituitarism or compression of the optic chiasm by mass effect | |
An aseptic meningitis is often seen | |
Usually asymptomatic. Where symptomatic, this presents as proximal weakness. Biopsy is reported to have a high diagnostic yield | |
Pure sensory and mixed neuropathies are reported. Mononeuritis multiplex is also described | |
Patients usually present non-specifically with a headache and visual disturbance. Clinical signs may include papilloedema | |
CSF and imaging show evidence of active inflammation, including meningeal enhancement and ventriculitis | |
Hydrocephalus may develop and may require surgical management | |
Can be a feature of cortical or subcortical disease | |
Mass lesions and inflammatory lesions are reported. A Guillain–Barré-like syndrome is occasionally described | |
In longitudinally extensive myelitis where aquaporin antibodies are negative, NS should be considered | |
Uveoparotid fever [13] | Uveitis, parotid gland swelling, fever and facial nerve palsy constitute this syndrome which is pathognomonic of sarcoidosis |
CSF often shows evidence of an aseptic meningitis | |
Ischaemic stroke, haemorrhagic stroke and dural venous sinus thrombosis are infrequently reported | |
Perivascular inflammation has been demonstrated in biopsy and post-mortem specimen |