Case 1 SF

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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.

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Other w/u

• LP elevated opening pressure, CSF lymphocytic
  pleocytosis (60-80), prt 80
• Serum ACE 65
• CSF ACE 20

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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

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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

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• 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

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Imaging
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Other w/u

• 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

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Clinical course

• IV steroids
• IVIG
• Mycophenolate
• Warfarin
• Worsening dementia and paraparesis
• D/c to SNF

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CNS Inflammatory Disease

• Primary, recurrent demyelinating diseases MS,
  Neuromyelitis Optica (NMO, Devics Dx)
• Mono-phasic demyelinating diseases Acute
  disseminated encephalomyelitis (ADEM), acute
  hemorrhagic leukoencephalitis (AHLE), transverse
  myelitis (TM), optic neuritis (ON) often these
  are para-infectious
• 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)

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Systemic inflammatory conditions with frequent
neurological manifestations

• SLE neuropsychiatric manifestations
• Sjogrens
• Sarcoid
• Anti-phospholipid Ab syndrome (1º or 2º)
• Rheumatoid arthritis PNS
• Vasculitis large or small vessel
• Large Giant cell arteritis CNgtCVA
• Small Wegeners, polyarteritis nodosum
  mononeuritis multiplex gt CN gtgtCNS
• Paraneoplastic syndromes cerebellar dx, limbic
  encephalitis, PNS

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Focal Clinical Presentation

• Focal CNS deficit (brain or brainstem)
  hemiparesis, hemisensory loss, hemiataxia,
  diplopia, vertigo, dysarthria
• Spinal cord syndrome complete (motor/sensory/auto
  nomic), anterior, posterior, Brown Sequard
• Cranial nerve optic neuritis, trigeminal
  neuralgia, facial paresis
• Pseudo-peripheral Lhermittes sign,
  paresthesias, pain
• Focal cognitive deficit aphasia, apraxia, neglect

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Neuropyschiatric SLE 19 syndromes described
Joseph (2007) Neurology
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NPSLE

• 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

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Neuro testing in NPSLE

• CSF abnl 20-40 (lymphocytic pleocytosis,
  elevated prt, OCB each present in 20).
• EEG abnl up to 80 abnl, mostly non-specific
  changes but some with epileptogenic focus.
• EMG/NCS high abnl in symptomatic PNS dx

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Neuroimaging

• Brain MRI abnl in 20-70 most common findings
  are multifocal small white matter
  hyperintensities and atrophy stroke in lt 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

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MRI abnl in NPSLE ptsCsepany (2003) J Neurol
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NPSLE RxSanna 2003
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CNS LupusCsepany 2003
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Lupus RPLSMagnano 2006
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EMT with SLE, APS, complicated migraine with
aphasia and RHP
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Neurosarcoidosis

• Neurological manifestations in 10 (20 at
  autopsy).
• Rarely presents with neurologic syndrome
• Very rarely limited to NS

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Joseph (2008) JNNP
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Spencer (2004) Sem Arthritis Rheum
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Laboratory findings in neurosarcoidosis

• 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

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Neurosarcoid MRI abnl

• Any abnl up to 80
• Leptomeningeal or parenchymal enhancement 25-50
  
• White matter lesions 30-50

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Neurological manifestations of Sjogrens 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 lt50 at presentation.

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PNS SjogrensMori (2005) Brain
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MRI, path, and sweat testing in Sjogrens sensory
neuropathy (Mori 2005)
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Lab abnl in Neuro-Sjogrens

• SSA/SSB 45
• Schirmers test abnl 90
• Salivary scintography abnl 65
• Lip bx abnl 95

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References

• SLE
• Joseph (2008) JNNP
• Sanna (2003) Lupus
• Csepany et al (2003) J Neurol
• Sjogrens
• Mori (2005) Brain
• Delalande (2004) Medicine
• Soliotis (1999) Ann Rheum Dis
• Sarcoid
• Joseph (2008) JNNP
• Joseph (2007) Practical neurology
• Spencer (2004) Sem Arthritis Rheum

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NeurosarcoidSpenser 2004
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CNS Sjogrens