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Neurosarcoidosis: Clinical Manifestations, Investigation and Treatment

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Neurosarcoidosis: Clinical Manifestations, Investigation and Treatment REVIEW Pract Neurol: first published as 10.1136/practneurol-2019-002349 on 17 May 2020. Downloaded from Neurosarcoidosis: clinical manifestations, investigation and treatment Desmond P Kidd Centre for Neurosarcoidosis, ABSTRACT eye may be involved, and skin, cardiac, Neuroimmunology unit, Sarcoidosis affects the nervous system in 10% hepatic, renal, bone and joint involve- Institute of Immunology and 3 Transplantation, University of cases. When it does so it can affect any part ment is common. College London, London, UK of the nervous system and with all degrees Around 30% of cases resolve within of severity. It forms part of the differential 2 years, particularly with single system Correspondence to Dr Desmond P Kidd, Royal Free diagnosis in inflammatory, infective, neoplastic involvement, 30% have a relapsing form Hospital, London NW3 2QG, UK; and degenerative neurological diseases and and 30% progressively deteriorate. d. kidd@ ucl. ac. uk may be very difficult to diagnose without histological confirmation. Recent clinical studies Accepted 5 January 2020 Epidemiology and the increasing availability of new biological Differences in the prevalence of the treatments allow a much clearer understanding disease have been known for decades; it of the disease. This review summarises its clinical has long been known that the incidence features, imaging and laboratory characteristics, is highest in Nordic countries 11–24 treatment and outcome. per 105,4 and lowest in south- east Asian countries (0.85 in Korea, 2.17 in Taiwan and 1.01 in Japan).5–8 African Americans INTRODUCTION within the USA have three times the inci- Sarcoidosis is an auto-inflammatory dence of European Americans,9 and the disorder characterised by the develop- incidence is lower still in Hispanic Amer- ment of granulomatous inflammation in icans.10 Recent studies from the Swedish affected tissues. Any tissue may become registry of sarcoidosis have identified affected, the most frequently being the 5 an annual incidence of 11 per 10 and a http://pn.bmj.com/ lungs, skin, eyes and liver. The lymphatic prevalence of 160 per 105,11 and in the system is always involved. At onset 95% USA three studies have shown an annual of cases have demonstrable lung involve- incidence of 11 per 105.12 In the UK ment, and half have multisystem disease1; the incidence was 14.5 per 105 between over time this prevalence increases in 1977 and 1983 on the Isle of Man,13 and those whose disease does not sponta- 5 recently 9.7 per 10 in a small study in on September 24, 2021 by guest. Protected copyright. neously remit.2 Hertfordshire, in which the prevalence of multisystem disease at onset was seen to Clinical features be 50%.14 The onset is subacute, rarely acute, with The age of onset varies, with very few constitutional symptoms including feeling cases occurring in children and the elderly, unwell, fever and night sweats before and a mean age of onset between 45 the heralding symptoms arise. Lofgren’s and 55 years, later in women than men. syndrome is the coexistence of fever, Overall it is more common in women arthritis of the ankles, erythema nodosum than in men.15 and hilar lymphadenopathy (figure 1). Over time the mortality rate is greater © Author(s) (or their employer(s)) 2020. No Heerfordt’s syndrome comprises a gran- than in the general population, around commercial re- use. See rights ulomatous uveitis, parotid and subman- 7%,16 relating predominately to the and permissions. Published dibular salivary gland swelling and cranial severity of the disease in those with respi- by BMJ. neuropathy (usually but not always the ratory, cardiac and neurological involve- To cite: Kidd DP. facial nerve). Lung involvement is charac- ment, but also to an increased risk of Pract Neurol Epub ahead of print: [please include Day terised by cough, breathlessness and chest infection. Patients with sarcoidosis also Month Year]. doi:10.1136/ tightness or stabbing pains, but is often have more other morbidities, such as practneurol-2019-002349 asymptomatic. Any structure within the venous thromboembolism, cardiovascular Kidd DP. Pract Neurol 2020;0:1–14. doi:10.1136/practneurol-2019-002349 1 REVIEW Pract Neurol: first published as 10.1136/practneurol-2019-002349 on 17 May 2020. Downloaded from examination, most have mild abnormalities of protein and cell count. Imaging is normal in half the cases, but in others shows nerve enhancement, and rarely shows more widespread changes in the absence of other clin- ical features.38 Optic neuropathy is common,20 38–41 arising through several different processes. A subacute optic neuritis is the most common and presents in an identical way to a demyelinating optic neuritis in most respects; pain is less prevalent, and the nadir acuity slightly less. Synchronous bilateral optic neuritis is uncommon, but sequential optic neuropathies occurred in 30%.41 Concurrent intraocular inflammation developed in 36% of one study.41 The associated field defects are central, centrocaecal or altitudinal. An optic perineuritis occurs when the optic nerve sheath becomes inflamed, leading to visual field constriction, disc swelling and pain. Chiasmal involve- ment is common when a basal leptomeningitis involves the hypothalamus and adjacent structures. Finally, Figure 1 (A) Chest X- ray showing Garland’s triad of bilateral a compressive optic neuropathy may arise when a symmetrical hilar lymphadenopathy and right para- tracheal dural inflammatory mass, usually at the orbital apex, lymphadenopathy. (B) H&E- stained section of mediastinal involves the optic nerve. There may also be sensory lymph node ×400 showing numerous well- formed granulomas loss and pain. and giant cells (courtesy Professor Peter Isaacson). (C) 18- FDG Imaging usually shows the causative abnormality positron- emission tomogram axial image through the chest with enhancement of the inflamed area (figure 2). The showing uptake within the mediastinal lymph nodes and the lung fields on both sides. cerebrospinal fluid (CSF) tends to be inactive or shows a mildly elevated CSF protein. Matched oligoclonal bands are uncommon; unmatched bands are not seen. disease and haematological and skin cancers,3 and a Isolated cranial neuropathies other than optic (II) higher prevalence of other autoimmune diseases, in and facial (VII) are less common but there are descrip- particular thyroid disease, connective tissue diseases tions of each nerve being involved; the oculomotor and multiple sclerosis.8 16 17 (III), trochlear (IV and abducens (VI) were involved relatively frequently in previous series, the trigeminal http://pn.bmj.com/ Neurological involvement (V) often alongside other nerves and the vestibuloco- 38 Accounts of neurological complications in the disease chlear (VIII) proportionately less frequently. Imaging began to be published as soon as it was recognised as is usually normal, but there may be nerve enhance- 42 a multisystem disease by Winckler in 1905,18 and by ment. Inflammatory masses within the orbit, at the Heerford 4 years later.19 A review in 1948 found 118 orbital apex and cavernous sinus may cause diplopia, 40 41 published cases, in which facial and optic neuropathies trigeminal sensory loss and pain, and proptosis, on September 24, 2021 by guest. Protected copyright. were seen most commonly, although any part of the and spread from an adjacent basal leptomeningitis may nervous system was affected.20 Later cohort studies of also involve these nerves. 285–807 patients attending systemic sarcoidosis clinics Isolated hearing loss is uncommon in sarcoidosis; it identified an incidence of neurological involvement occurs suddenly, often alongside a vestibular syndrome, 43 44 23 of 3.5%–7%.19–23 More recent case series comprising progressively deteriorates and may fluctuate. 43 19–54 cases21–37 showed that around half of patients Corticosteroids may rescue hearing but most do not have isolated cranial neuropathies, of which the facial recover. Imaging is either normal or shows a pachy- nerve is by far the most common, with peripheral meningeal thickening within the internal auditory 43 44 nerve involvement in 10% and muscle involvement in canal. Spread from mastoid sinuses into the middle 5%, and with brain and spinal cord lesions accounting ear may also occur. The vestibulocochlear nerve may for the remainder; 16%–70% of these series. The be involved as part of a spreading pachymeningitis or prevalence of neurological complications in one series a leptomeningitis in the region, often with accompa- from Japan was 7.2%.7 nying facial nerve palsy. Isolated neuropathies of the lowest cranial nerves Cranial neuropathy are uncommon, but I have often seen a bulbar disorder Facial neuropathy accounts for 70% of isolated cranial characterised by progressive dysphonia and dysphagia, neuropathy. Most are unilateral and occur at the onset although this is infrequent in the literature.20 Patients of the disease. Among those undergoing a spinal fluid in my series were all female, and most had normal 2 Kidd DP. Pract Neurol 2020;0:1–14. doi:10.1136/practneurol-2019-002349 REVIEW Pract Neurol: first published as 10.1136/practneurol-2019-002349 on 17 May 2020. Downloaded from Figure 2 Cranial neuropathy in neurosarcoidosis. (A, B) Left optic neuropathy; high signal within the nerve with associated enhancement. (C) Enhancement of the right third nerve. (D) Enhancement of the left seventh nerve. (E, F) Enhancement of the left seventh and eighth nerves. imaging but slightly active CSF.38 Occasionally there infiltration of the epineurium with a perivascular is weakness and atrophy of one side of the tongue.20 infiltration of inflammatory cells and granulomas Again, involvement of these nerves may form part of around the nerve (figure 3). There may be nerve fibre a more widespread disorder due to a basal meningitis. disorganisation and axonal loss.45 46 53 54 Pathological features of a perineural vasculitis occurred in 40% of Peripheral neuropathy Professor Said’s cases. Other authors have also noted Peripheral neuropathy accounts for 10%–14% of that the neuropathy develops years after the onset of http://pn.bmj.com/ some series, but much less in others.
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