How should I structure a neuro‑ICU consult note, including assessment, plan, and orders, for a 50‑year‑old female with systemic lupus erythematosus off therapy for seven months and antiphospholipid syndrome who presents with sub‑acute encephalopathy, Glasgow Coma Scale 10, inflammatory cerebrospinal fluid (elevated white blood cells, markedly high protein, normal glucose) and magnetic resonance imaging showing multifocal acute diffusion‑restricted lesions, chronic deep white‑matter and basal‑ganglia changes, mild leptomeningeal enhancement, and a negative computed tomography angiography for large‑vessel occlusion?

Neuro-ICU Consult Note Structure for Severe Neuropsychiatric SLE with APS

Assessment

This is a life-threatening neuropsychiatric SLE flare with inflammatory small-vessel vasculopathy and multifocal acute ischemia in the setting of untreated disease and antiphospholipid syndrome. 1

Primary Diagnosis

• Neuropsychiatric SLE (NPSLE) with acute confusional state and probable CNS vasculitis
  • Subacute encephalopathy progressing to GCS 10 1
  • Inflammatory CSF (WBC 24, protein 3.2 g/L) consistent with NPSLE, not typical infection 1
  • Multifocal acute diffusion restriction on MRI indicating active ischemic injury 1
  • Extensive chronic white matter and basal ganglia disease reflecting prior vasculopathy 1
  • Mild leptomeningeal enhancement supporting inflammatory process 1

Contributing Diagnosis

• APS-related microthrombotic disease
  • Known APS with prior CVA 2, 3
  • Multiple acute infarcts despite negative CTA for large vessel occlusion 1, 2
  • Pattern consistent with small vessel thrombosis superimposed on inflammatory vasculopathy 3, 4

Differential Considerations

• Infectious meningoencephalitis (HSV/VZV) - must exclude with pending CSF PCR 1
• Septic encephalopathy - less likely given inflammatory CSF pattern but cannot exclude 1

Immediate Management Plan

1. Dual-Track Approach: Treat Infection AND Inflammation Simultaneously

Continue empiric antimicrobial coverage until infection definitively excluded 1:

• Acyclovir for HSV/VZV coverage (continue until CSF PCR negative)
• Broad-spectrum antibiotics (continue until cultures negative and clinical picture clarifies)

Aggressive immunosuppression already initiated appropriately 1, 5:

• IV methylprednisolone 1000 mg daily × 3 days (pulse therapy) 1, 5
• This is correct per EULAR guidelines: high-dose glucocorticoids should be given early even while awaiting infection exclusion in inflammatory CSF 1

2. Escalate to Combination Immunosuppression Within 24-48 Hours

Add IV cyclophosphamide immediately after infection reasonably excluded 1, 5:

• Dose: 500-750 mg/m² IV (single dose, then monthly) 1, 6
• EULAR guidelines state that combination of IV methylprednisolone and IV cyclophosphamide is effective in severe NPSLE, including acute confusional state (70% response rate) and must be used promptly 1, 5
• Timing is critical: delay >2 weeks associated with worse neurological outcomes 1, 5

Alternative if cyclophosphamide contraindicated:

• Rituximab has shown rapid improvement in refractory NPSLE 1
• Consider if bone marrow intolerance or patient preference 6

3. Anticoagulation Strategy for APS

Continue therapeutic anticoagulation 1, 2:

• Current enoxaparin 1 mg/kg BID is appropriate for acute phase 7
• Transition to warfarin for long-term secondary prevention 1, 2
  • Target INR 2.0-3.0 for venous thrombosis 7
  • Consider INR 3.0-4.0 if recurrent arterial events despite therapeutic anticoagulation 1
• EULAR guidelines state anticoagulation may be superior to antiplatelet therapy for secondary prevention of arterial events (including stroke/TIA) in APS 1

4. Complete Diagnostic Workup

CSF studies (ensure sent) 1:

• HSV-1, HSV-2, VZV PCR (pending - critical)
• Bacterial/fungal cultures (pending)
• Oligoclonal bands (assess for intrathecal inflammation)
• Consider autoimmune encephalitis panel if available

Serum studies:

• Complement levels (C3, C4) to assess disease activity
• Anti-dsDNA, anti-Smith antibodies
• Complete antiphospholipid antibody panel if not recent (lupus anticoagulant, anticardiolipin IgG/IgM, anti-β2-glycoprotein I IgG/IgM) 2
• Anti-ribosomal P antibodies (limited utility but may support lupus psychosis if present) 1

Neurophysiology:

• Urgent EEG to exclude nonconvulsive status epilepticus 1
• GCS drop mandates this evaluation 1

Advanced imaging:

• Consider repeat MRI in 7-10 days to assess treatment response 1, 5
• Vessel wall imaging if available to confirm vasculitis 4, 8

5. Neuroprotective and Supportive Care

ICU-level monitoring 1:

• Hourly neurological assessments
• Maintain airway protection (consider intubation if GCS drops further or unable to protect airway)
• Strict blood pressure control (avoid hypotension and hypertensive surges)
• Normoglycemia (tight glucose control)
• Avoid hyperthermia

Seizure prophylaxis consideration:

• Do NOT routinely start antiepileptic drugs without documented seizures or epileptiform activity on EEG 1
• If seizures occur: treat with standard AEDs plus intensify immunosuppression if concurrent lupus activity 1

Infection prophylaxis:

• Consider antifungal prophylaxis (fluconazole or alternative) given high-dose steroids 5
• PCP prophylaxis (trimethoprim-sulfamethoxazole or alternative) once cyclophosphamide started

6. Refractory Disease Planning

If no improvement within 3-5 days or clinical deterioration 1, 6:

• Add plasmapheresis (5 exchanges over 7-10 days, synchronized with cyclophosphamide) 1, 6
• Consider IV immunoglobulin 400 mg/kg/day × 4-5 days 6
• Escalate to rituximab if above measures fail 1

Critical Orders

Immediate (within 1 hour):

• Continue IV methylprednisolone 1000 mg daily × 3 days (day 2 of 3)
• Continue acyclovir IV (dose per renal function)
• Continue broad-spectrum antibiotics
• Continue enoxaparin 1 mg/kg SC BID
• Urgent EEG (portable if unstable)
• Neurology checks q1h with GCS documentation
• NPO status (aspiration precautions given altered mental status)

Within 24 hours:

• Rheumatology consultation for cyclophosphamide timing
• Infectious disease consultation for antimicrobial stewardship
• Expedite CSF PCR results (call lab)
• Send additional serum studies (complement, anti-dsDNA, antiphospholipid panel)
• Pharmacy: prepare cyclophosphamide 500-750 mg/m² IV (hold pending infection exclusion)
• Fluconazole 400 mg daily (antifungal prophylaxis)

Within 48-72 hours:

• Administer IV cyclophosphamide once HSV PCR negative and bacterial cultures showing no growth at 48 hours 1, 5
• Transition to oral prednisone 1 mg/kg/day (max 60 mg) after pulse therapy complete 5
• Initiate warfarin overlap with enoxaparin (target INR 2.0-3.0) 7
• Repeat MRI brain with contrast in 7-10 days to assess response 1, 5

Prognostic Factors and Monitoring

Favorable response indicators 1, 5:

• Neurological improvement should parallel MRI improvement within days to 3 weeks 1, 5
• GCS improvement within 48-72 hours of pulse steroids suggests inflammatory etiology responding to treatment 1

Poor prognostic factors 1, 2:

• Delay in treatment initiation >2 weeks 1, 5
• Extensive MRI lesions 1
• Triple antiphospholipid antibody positivity (highest thrombotic risk) 2
• Persistently positive moderate-to-high titer antiphospholipid antibodies 2

Relapse risk 1, 5:

• 50-60% relapse rate during steroid taper without adequate maintenance immunosuppression 1, 5
• Mandatory maintenance therapy after induction: azathioprine 2 mg/kg/day or mycophenolate mofetil 2-3 g/day 1, 6
• Continue for minimum 2 years, possibly longer 6

Common Pitfalls to Avoid

Most dangerous error: Attributing all symptoms to lupus without adequately excluding infection 2:

• This patient is off treatment × 7 months and now on high-dose steroids
• Must definitively exclude HSV encephalitis before declaring this pure NPSLE 1

Second critical error: Delaying cyclophosphamide 1, 5:

• Once infection excluded, every hour of delay worsens neurological prognosis 1, 5
• Do not wait for "more data" - act within first 24-48 hours 1, 5

Third error: Inadequate anticoagulation in APS 1, 2:

• This patient has documented APS with prior stroke and now multifocal infarcts 2
• Antiplatelet therapy alone is insufficient for secondary prevention 1
• Must transition to therapeutic warfarin 1, 7

Fourth error: Stopping immunosuppression too early 1, 5:

• Relapses are extremely common (50-60%) without maintenance therapy 1, 5
• Plan for 2+ years of maintenance immunosuppression from the outset 6

Fifth error: Normal labs do not exclude active CNS lupus 2:

• Neurological involvement can occur independently of systemic disease activity 7, 2
• Do not be falsely reassured by normal complement or negative anti-dsDNA 2