Case and Discussion: Chronic and Acute Confusional States

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Case and DiscussionChronic and Acute
Confusional States

• Connie Chen, MD
• Neurology Consultants of Dallas

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Overview

• Case presentation
• Differential diagnosis
• Clinical approach
• Results and findings
• Follow-up
• Discussion

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Case Presentation

• 61 yo woman
• episode of presyncope
• wobbly when standing
• slow thinking over 6 months
• noted after administration of BP meds (SBP 200s
  lowered to 120s)
• NRO exam non-focal. MS not extensively tested,
  some memory loss noted
• Hyponatremic Na117

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Case Continued

• Diuretic stopped
• BP raised slightly
• PT d/cd to home after Na normalized

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Case Continued

• 2 weeks later
• Episodic worsening of confusion
• Lost while driving
• Worsening short-term memory
• Episodes of paranoia
• New delusions
• CT scanner trying to transport her to the future
• Aliens trying to abduct daughter
• After watching Manchurian Candidate, she was
  also involved in a conspiracy

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Case Continued

• NRO exam
• MS
• Poor memory, attention, not oriented
• Labile affect
• Intact calculations, language
• Delusional
• CN, motor, sensation, cerebellar, and gait are
  normal

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Differential diagnosis Chronic confusional state

• Progressive decline of memory, cognition
• Degenerative dementias
• Multi-infarct dementia
• Chronic infection (TB meningitis, syphilis, HIV)
• Hypothyroidism
• Vitamin deficiencies (B12, thiamine)
• Toxins
• Other seizures, neoplastic, paraneoplastic,
  pseudo-dementia

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Differential diagnosis Acute confusional state

• Delirium
• Metabolic states
• Medications/drugs
• Endocrine thyroid, glucose, hyper/hypoadrenalism
  
• Electrolytes Na, Ca
• Vitamins B12, thiamine
• Organ failure liver, renal (uremia, dialysis
  disequilibrium), respiratory failure (hypoxia)

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Acute Confusional State

• Cerebrovascular
• stroke/TIA
• hypertensive encephalopathy, hypotension
• DIC, TTP
• Infectious meningitis
• Seizures
• Head trauma
• Neoplasm
• Other (Systemic disease rheumatologic,
  paraneoplastic)

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Clinical approach

• Systematic approach
• Indications for studies
• Dont stop with one diagnosis
• Think outside the box
• What am I missing?
• Tailor your work-up, you can always expand later

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Our case Results and Findings

• Chronic confusional state (gt6 month decline)
• Degenerative dementias
• Diagnosis of exclusion
• Requires memory loss in addition to another
  cognitive sphere with functional decline
• Multi-infarct dementia no evidence of
  infarction.
• Chronic infection LP negative ( mild protein
  elevation), RPR negative, HIV negative.
• Hypothyroidism nl TSH
• Vitamin deficiencies (B12, thiamine) low B12,
  normal homocysteine
• Toxins negative tox screen

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Results Continued

• Acute confusional state
• Metabolic
• Meds none
• Endocrine TSH normal, normo-glycemia
• Infections LP negative except elevated protein,
  RPR negative, HIV negative.
• Vitamins B12 low but homocysteine normal (MMA
  pending), thiamine given.
• Electrolytes Na 131, dropped to 127.
• Organ failure organs normal, no respiratory
  failure.

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Results Continued

• Cerebrovascular no focality to suggest
  stroke/TIA, not hyper or hypotensive, no evidence
  DIC/TTP.
• Seizure left temporal sharp wave. No seizure.
• Neoplasm normal head CT.

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What else am I missing?

• Delirium with new onset pyschosis
• Antiphospholipid antibody syndrome
• Limbic encephalitis (paraneoplastic syndrome)
• Porphyria

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More Results

• ESR, ANA, anticardiolipin antibodies negative.

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More Results

• Chest CT
• right paratracheal node
• 0.8 cm nodular opacity right upper lobe.
• Biopsy of node small cell lung cancer.

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Follow-up

• Treatment with XRT and CMTx.
• Psychotic symptoms resolved.
• Memory loss remains.

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Discussion

• Limbic encephalitis
• a paraneoplastic syndrome marked by
  degeneration of neurons in the medial temporal
  lobe.

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Limbic encephalitis

• Incidence unknown (rare)
• Symptoms
• Acute confusional states
• Memory loss
• Seizures
• Psychiatric symptoms
• Dementia
• Antineuronal antibodies anti-Hu, anti-Ta,
  (anti-Ma, others)

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Limbic encephalitis

• Often presents before tumor diagnosis
• Tumor associations
• Lung (small cell, non-small cell)
• Testicular
• Breast

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Limbic encephalitis Studies

• EEG temporal lobe seizures, sharp waves, normal.
• CSF (can be normal)
• Mild pleocytosis
• Mildly elevated protein
• Radiographic
• MRI (can be normal)
• medial temporal lobe bright on T2, enhances
  with contrast.
• brainstem
• hypothalamus
• r/o HSV encephalitis

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Limbic encephalitisTreatment

• Treatment underlying tumor
• Immune modulatory treatments attempted
• Steroids
• Cyclophosphamide
• IV IG
• Plasmapheresis
• Improvement of symptoms only with tumor treatment
• If diagnosed- search for tumor!