Case 1 SF
• 41 y/o woman with no PMHx p/w blurry
vision and headache worsening x few
weeks. No meds, no toxic habits.
• PE: Afeb VSS. Bilateral lid swelling.
• Neuro exam: MSE with mild cognitive
slowing and diminished attention,
otherwise normal. CN: diminished VA with
disc swelling b/l.
• Motor/sensory/cerebellar intact.
Other w/u
• LP: elevated opening pressure, CSF
lymphocytic pleocytosis (60-80), prt 80
• Serum ACE: ~65
• CSF ACE: ~20
Clinical course
• Progressive cognitive decline, recurring
bouts of aseptic meningitis with visual
blurring partially responsive to IV steroids.
• Poor compliance pulse Cytoxan
• Later developed poor vision d/t glaucoma
and b/l optic neuritis
• Panhypopituitarism
• Dementia
Case 2 RC
• 44 y/o man with hx of CVA and behavioral
problems, p/w AMS and difficulty walking.
Prior CVA admitted at Montefiore 2005,
p/w . W/u revealed basal ganglia and
cerebellar calcification on CT scan, acute
R midbrain/thalamic infarct on MRI.
Cause of stroke in young uncertain,
however pt found to be ANA+.
• Meds:
• PE: Afeb VSS
Neuro: MSE: alert, Ox2, dim attention, STM
1/3.
CN: dysarthric, otherwise intact
Motor: increased tone in legs, full strength,
mild incoordination on FTN,
DTRs: hyperactive
Gait: spastic/ataxic
Imaging
Other w/u
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ESR 120
Creatinine 2-3 (prior baseline 1-1.5)
ANA and dsDNA+
Anti-cardiolipin Ab+
MRI spinal cord: no significant abnl
Cerebral angiogram: possibly slight
medium vessel irregularity c/w
vasculopathy
Clinical course
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IV steroids
IVIG
Mycophenolate
Warfarin
Worsening dementia and paraparesis
D/c to SNF
CNS Inflammatory Disease
• Primary, recurrent demyelinating diseases: MS,
Neuromyelitis Optica (NMO, Devic’s Dx)
• Mono-phasic demyelinating diseases: Acute
disseminated encephalomyelitis (ADEM), acute
hemorrhagic leukoencephalitis (AHLE), transverse
myelitis (TM), optic neuritis (ON); often these are parainfectious
• CNS involvement with systemic (clinical or sub-clinical)
auto-immune disease; includes primary and secondary
CNS vasculitis
• Paraneoplastic dx
• Immune reconstitution inflammatory syndrome (IRIS)
• CNS infections (discussed in other lecture)
Systemic inflammatory conditions with
frequent neurological manifestations
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SLE neuropsychiatric manifestations
Sjogren’s
Sarcoid
Anti-phospholipid Ab syndrome (1º or 2º)
Rheumatoid arthritis: PNS
Vasculitis: large or small vessel
Large: Giant cell arteritis: CN>CVA
Small: Wegener’s, polyarteritis nodosum: mononeuritis
multiplex > CN >>CNS
• Paraneoplastic syndromes: cerebellar dx, limbic
encephalitis, PNS
Focal Clinical Presentation
• Focal CNS deficit (brain or brainstem):
hemiparesis, hemisensory loss, hemiataxia,
diplopia, vertigo, dysarthria
• Spinal cord syndrome: complete
(motor/sensory/autonomic), anterior, posterior,
Brown Sequard
• Cranial nerve: optic neuritis, trigeminal
neuralgia, facial paresis
• Pseudo-peripheral: Lhermitte’s sign,
paresthesias, pain
• Focal cognitive deficit: aphasia, apraxia, neglect
Neuropyschiatric SLE:
19 syndromes described
Joseph (2007) Neurology
NPSLE
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Neurological dx present in: ~50% (15-90%)
Presenting with neuro symptoms: 3-5%
NPSLE worsens prognosis
NPSLE can occur without systemic flare
Lab abnl: ESR elevated 50%, ANA+ 85%,
dsDNA+ 72%, anti-phospholipid Ab 30%,
complement low during flare 44%, ribosomal P
Ab and C3A frequently elevated prior to/during
flare.
• APS associated with NPSLE, CVA, other focal
dx
Neuro testing in NPSLE
• CSF abnl: 20-40% (lymphocytic
pleocytosis, elevated prt, OCB each
present in ~20%).
• EEG abnl: up to 80% abnl, mostly nonspecific changes but some with
epileptogenic focus.
• EMG/NCS: high% abnl in symptomatic
PNS dx
Neuroimaging
• Brain MRI: abnl in 20-70%; most common
findings are multifocal small white matter
hyperintensities and atrophy; stroke in <
20%; lower % show basal ganglia
calcification, reversible
leukoencephalopathy syndrome (RPLS).
• SPECT: detects multifocal or
patchy/diffuse perfusion deficits in 50-90%
• MR spectroscopy: abnl in ? 20-50%
MRI abnl in NPSLE pts
Csepany (2003) J Neurol
NPSLE Rx
Sanna 2003
CNS Lupus
Csepany 2003
Lupus RPLS
Magnano 2006
EMT with SLE, APS, complicated
migraine with aphasia and RHP
Neurosarcoidosis
• Neurological manifestations in ~10%
(~20% at autopsy).
• Rarely presents with neurologic syndrome
%
• Very rarely limited to NS %
Joseph (2008) JNNP
Spencer (2004) Sem Arthritis Rheum
Laboratory findings in
neurosarcoidosis
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CXR abnl: ~40-50% (30-80% range)
Chest CT abnl: ~60-75% (? up to 90%)
Gallium/PET scan abnl: 25-80%
Serum ACE elevation: 25-75%
CSF prt elevation: 50%
CSF lymphocytic pleocytosis: 40%
CSF OCB: 20-40%
Neurosarcoid MRI abnl
• Any abnl: up to 80%
• Leptomeningeal or parenchymal
enhancement: 25-50%
• White matter lesions: 30-50%
Neurological manifestations of
Sjogren’s syndrome
• Common disorder, affecting ~2-3% of
adults.
• Neurological dx present in 5-60%.
• CNS and PNS dx both common.
• Neurological symptoms occur prior to
diagnosis in 80-90% of patients.
• Sicca symptoms present in <50% at
presentation.
PNS Sjogren’s
Mori (2005) Brain
MRI, path, and sweat testing in Sjogren’s
sensory neuropathy (Mori 2005)
Lab abnl in Neuro-Sjogren’s
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SSA/SSB+: 45%
Schirmer’s test abnl: 90%
Salivary scintography abnl: 65%
Lip bx abnl: 95%
References
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SLE:
Joseph (2008) JNNP
Sanna (2003) Lupus
Csepany et al (2003) J Neurol
Sjogren’s:
Mori (2005) Brain
Delalande (2004) Medicine
Soliotis (1999) Ann Rheum Dis
Sarcoid:
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Joseph (2008) JNNP
Joseph (2007) Practical neurology
Spencer (2004) Sem Arthritis Rheum
Neurosarcoid
Spenser 2004
CNS Sjogren’s