Neuro-immunology AKA Neurologic manifestations of autoimmune diseases

44 Pages·2016·0.68 MB·English

Neuroimmune Disorders
AKA
Neurologic manifestations of autoimmune
diseases
Bridget T Walsh, DO
Catalina Pointe Arthritis & Rheumatology Specialists, PC
Objectives
•
Review neurologic conditions that have an
underlying autoimmune etiology
•
Discuss initial diagnostic work-up to determine
what could be causing the neurologic symptoms
•
Review neurologic manifestations of specific
autoimmune diseases that I care for
•
Discuss briefly treatment of neurologic
manifestations of specific autoimmune diseases
•
Review briefly the most common autoimmune
neurologic diseases (GBS, MS, MG)
Disclaimer
I am a rheumatologist not a neurologist or a
magician so…..I will do my best and try to focus
on a more primary care approach to these
neurologic problems.
Immune-mediated neuropathies:
Chronic
Acute
1. Multiple Sclerosis
• Guillain-Barré syndrome 2. Myasthenia gravis
(GBS)-is an acute monophasic 3. Chronic inflammatory demyelinating
illness resulting from an polyneuropathy (CIDP) (prototype)
immune response to a
4. Multifocal motor neuropathy
preceding infection that cross-
(MMN)
reacts with peripheral nerve
5. POEMS syndrome (osteosclerotic
components because of
myeloma: polyneuropathy,
molecular mimicry. The
organomegaly, endocrinopathy,
immune response can be
monoclonal protein, skin changes)
directed towards the myelin or
6. Anti-myelin associated glycoprotein
the axon of peripheral nerve,
(MAG)-related neuropathies
resulting in demyelinating and
axonal forms of GBS. Note: # 4,5 &6 are distinct from classic
CIDP on the basis of clinical,
electrodiagnostic, and therapeutic
differences.
What did I get myself into?
Other possible neuroimmune
syndromes
Those w/ likely or possible Systemic disorders that may be
immune mediated mechanism associated w/ neuroimmune Dx
• Classic, idiopathic CIDP • Monoclonal gammopathy of
• Multifocal acquired demyelinating undetermined significance
sensory and motor neuropathy
(MGUS)
(MADSAM) or Lewis–Sumner syndrome
• Chronic immune sensory • Multiple myeloma
polyradiculopathy (CISP)
• Waldenstrom
• Sensory-predominant CIDP
macroglobulinemia
• Distal acquired demyelinating
• Mixed cryoglobulinemia
symmetric (DADS) neuropathy
• Demyelinating neuropathy with central • Systemic disorders (eg,
nervous system demyelination
diabetes mellitus,
• Gait ataxia with late onset
thyrotoxicosis, neoplasms, SLE,
polyneuropathy (GALOP) syndrome
RA, Sjogren’s, systemic
• Multifocal motor neuropathy
vasculitides,
• Neuromyelitis Optica (mixed axonal and
demyelinating)
So where to begin?
Presenting symptoms
(Important but poor diagnostic accuracy)
Sensory Motor
• Numbness/tingling • Weakness
• Burning – Symetric vs asymetric
– Proximal vs distal
• Ants crawling
• Balance
• Pain/Lancinating
Dysautonomia
Respiratory
Ophthalmoplegia
Bulbar
Callaghan B et al, Review JAMA 2015;314:2172; England JD et al, Neurology
2005;64:199; Neurology 2009;72:186; England JD, Asbury AK Lancet 2004;363:2151;
Rutkove SB, Overview of polyneuropathy, Up to date Sept 2016, Shefner JM Ed.
Physical Exam Findings-
Better predictor than Symptoms
(especially if > 1 abnormality)
Sensory exam Motor Exam
• Distal sensation • Strength
• Pin Prick / Light touch/Temp • Atrophy?
• Vibratory • Fasciculations?
• Position
Autonomic
• DTRs/Babinski
- Postural hypotension
- Impotence
- Bladder dysfunction)
Mendell JR, Sahenk Z, NEJM, 2003;348:1243
Rutkove SB Up to Date, Sept 2016 , Shefner JM ed.
Electro-diagnostic Testing
EMG/NCS
Demyelinating
Axonal Disorders
Radicular, denervation/ reinnervation, Neuromusclular Junction
Labs-
Ideally should be based on
Electro-diagnostic testing
Other:
CSF, Imaging, Genetics, Tissue biopsy
Callaghan B et al, Review JAMA 2015;314:2172; England JD et al, Neurology 2005;64:199;
Neurology 2009;72:186; England JD, Asbury AK Lancet 2004;363:2151; Rutkove SB,
Overview of polyneuropathy, Up to date Sept 2016, Shefner JM Ed Great Algorithm.