Content Other autoimmune CNS disease Philippe Demaerel Vasculitis Systemic lupus erythematosus Wegener granulomatosis Behçet disease Rhombencephalitis - CLIPPERS Neurosarcoidosis Langerhans cell histiocytosis HLH Susac syndrome Rasmussen encephalitis Parry Romberg syndrome Tumefactive demyelination Vasculitis Polyarteritis nodosa Headache, seizure, subcute cognitive decline. Primary CNS angiitis. Connective tissue disorders and systemic vasculitis (lupus, Behçet, Sjögren). CAA Aß-related angiitis, CAA related inflammation. Giant cell arteritis, Wegener, Takayasu DD. : RCVS Primary cerebral angiitis Giant cell arteritis
Giant cell arteritis Systemic lupus erythematosus Cortical atrophy. Infarcts: large, lacunar, border zone. Demyelinating syndrome. Vessel wall lesions in large intracranial arteries ( imaging): eccentric wall thickening. ( Sneddon syndrome Livedo reticularis and cerebrovascular disease
Wegener granulomatosis Granulomatosis with polyangiitis. Direct or remote granulomatous invasion from contiguous lesions in the nose and paranasal sinuses, Headache, cranial nerve palsy, pachymeningitis (DD. neurosarcoidosis, IgG4 disease). Behçet disease Oral and genital ulcers, uveitis. Cranial nerves, brainstem and basal ganglia. Three categories: - Brainstem syndrome and systemic signs (fever and arthralgia). - Meningomyelitis. - Confusional syndrome and meningoencephalitis. Rhombencephalitis Infectious Autoimmune Paraneoplastic GBS: demyelination in the peripheral nervous system Bickerstaff: disturbances of consciousness and pyramidal signs: anti GQ1b Ab. Miller-Fischer: external ophthalmoplegia and ataxia. CLIPPERS
CLIPPERS Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Gadolinium enhancing nodules without ring enhancement in pons and cerebellum. High T2/FLAIR signal area not exceeding the area of enhancement. Anti-MOG antibodies? Neurosarcoidosis 5-16% of sarcoidosis 1. Systemic sarcoidosis and neurological symptoms: high suspicion, DD. immunesuppression for sarcoidosis. If evidence of neuroinflammation: very high suspicion. 2. Typical syndrome: uveo-parotid syndrome, cranial oligoneuropathy, spinal cord LETM, cauda equina, hypothalamus-pituitary gland. 3. Cryptogenic neuroinflammation, after exclusion of common infectious and autoimmune disorders. Polymyalgia rheumatica
Idiopathic hypertrophic pachymeningitis Rheumatoid meningitis Langerhans cell histiocytosis - HLH LCH orbit, mastoid, temporal bone and diabetes insipidus predispose to CNS-LCH. CD8+ lymphocytes infiltrate the brain with production of cytokines or Ab with direct effect on neurons. CNS-LCH: - Neurodegenerative syndrome: ataxia, dysarthria and behavioural changes. - Mass lesions. Hemophagocytic Lympho-Histiocytic syndrome: hyperinflammation caused by uncontrolled proliferation of lymphocytes and histiocytes. Hemophagocytic Lympho-Histiocytosis Erdheim Chester disease Non-Langerhans cell histiocytosis. Always skeletal involvement. 50% extraskeletal: CNS: brainstem, cerebellum and pituitary gland. Thickened facial bones with osteosclerosis.
Susac syndrome Encephalopathy, visual disturbances, hearing loss Small arteries of brain, inner ear and retina White and grey matter Small circular lesion centrally in the corpus callosum Leptomeningeal enhancement (30%) Chronic stage: spoke-like and atrophy Rasmussen encephalitis (RE) Chronic inflammation (T-cell response to antigene) with progressive brain destruction over several weeks/years. Perisylvian region is predilection site. Intractable seizures and progressive hemiplegia. Stage 1: swelling and increased signal. Stage 2: normal volume and increased signal. Stage 3: Atrophy and increased signal. Stage 4: Atrophy and normal signal. Kuchling J, Sinnecker T, Bozin I, Dörr J, Madai VI, Sobesky J, Niendorf T, Paul F, Wuerfel J. Ultrahigh field MRI in context of neurological diseases. Nervenarzt. 2014 Apr;85(4):445-58
Diagnostic criteria RE Part A (all three criteria): - Clinical signs - EEG - Brain MR: unilateral focal cortical atrophy and at least one of the following: (a) Increased signal in the gray or white matter, (b) increased signal or atrophy of the caudate head. Part B (2/3 criteria): - Clinical signs - Brain MR: progressive unilateral focal cortical atrophy - Histopathology 11.2012 01.2013 01.02.2013 11.02.2013 04.2013 11.2013 stage 1 stage 2 stage 3 07.2013 07.2013 08.2013 07.2013 stage 4 Parry Romberg syndrome Progressive facial hemiatrophy (skin to bone) Similarities with linear scleroderma en coup de sabre. ANA, cardiolipin, phospholipid Ab. Crossed cerebellar diaschisis.
Tumefactive demyelination (TD) Solitary locally aggressive demyelination. TD-MS, TD-CIS, TD-SLE, Behçet, TD-tacrolimus. TD-paraneoplastic: close temporal relationship of demyelination and malignancy, e.g. germ cell tumor, renal cell carcinoma, lymphoma. MR imaging: periventricular and deep white matter, open ring enhancement, little mass effect, decreased perfusion, peripheral diffusion restriction. Conclusion Vasculitis can adequately be imaged at 3T. The role of vessel wall imaging will increase in autoimmune diesease and is to be obtained together with MR angiography. Brainstem, cerebellar, pituitary, lepto- and pachymeningeal pathology are common sites of involvement in autoimmune diseases. Susac syndrome can easily be differentiated from MS. MR imaging plays a role in diagnosing Rasmussen encephalitis/parry Romberg syndrome. Tumefactive demyelination can occur in an autoimmune setting.