How to write the cause of death in cases of New-Onset Refractory Status Epilepticus (NORSE) according to Indian rules or standards?

Death Certification in NORSE Cases

In cases of New-Onset Refractory Status Epilepticus (NORSE), the cause of death should be documented as the immediate physiological mechanism of death (e.g., cardiorespiratory arrest, cerebral edema, multi-organ failure) as Part I(a), with "New-Onset Refractory Status Epilepticus" listed as the underlying condition in Part I(b), and any identified etiology (e.g., autoimmune encephalitis, infection) in Part I(c) if determined.

Understanding NORSE as a Cause of Death

NORSE represents a devastating neurological emergency with mortality rates of 12-27% in the acute phase, and these rates can increase substantially in refractory cases 1. The condition is characterized by explosive-onset refractory status epilepticus without evident etiology or active epilepsy, often progressing to super-refractory status epilepticus (SRSE) 1.

Death Certificate Structure for NORSE

Following standard medical certification principles (adapted from cardiovascular death certification frameworks), the death certificate should follow a logical sequence from immediate cause to underlying condition 2:

Part I - Chain of Events Leading to Death:

• Line (a) - Immediate cause: Document the terminal physiological event

  • Examples: "Cardiorespiratory arrest," "Cerebral edema with herniation," "Multi-organ failure," "Refractory hypoxia" 1

• Line (b) - Intermediate cause: "New-Onset Refractory Status Epilepticus (NORSE)"

  • This should always be listed as it represents the underlying neurological condition 1

• Line (c) - Underlying cause (if identified): Document the etiology when determined

  • "Autoimmune encephalitis" (most common identified cause) 2, 1
  • "Cat-scratch disease (Bartonella henselae infection)" 3
  • "Suspected organophosphate toxicity" 4
  • "Cryptogenic" or "Unknown etiology" if no cause identified (occurs in approximately 50% of adult cases) 1

Part II - Other Significant Conditions:

• List complications that contributed but were not directly in the causal chain
  • Examples: "Nosocomial pneumonia," "Sepsis," "Acute kidney injury" 1

Specific Documentation Considerations

When Etiology is Identified

Autoimmune encephalitis is the most frequently identified cause of NORSE 1. When confirmed through antibody testing or histopathology, this should be documented:

• If anti-GAD antibodies, anti-NMDAR antibodies, or other specific antibodies are identified, specify these 2, 5
• If histopathological evidence exists (vasculitis, necrotizing vasculopathy, lymphocytic infiltration), reference the pathology findings 5

When Etiology Remains Unknown

Approximately 50% of adult NORSE cases remain cryptogenic despite extensive workup 1. In these cases:

• Use "New-Onset Refractory Status Epilepticus of unknown etiology" or "Cryptogenic NORSE" 1
• Do not leave the underlying cause blank, as NORSE itself represents a specific clinical syndrome requiring documentation 1

Common Pitfalls to Avoid

Do not list only "seizures" or "status epilepticus" without specifying NORSE, as this fails to capture the unique clinical entity and its implications for mortality and morbidity 1.

Do not omit the immediate physiological mechanism of death. While NORSE is the underlying condition, death certificates require documentation of the terminal event (e.g., respiratory failure, cardiac arrest) 2.

Avoid vague terminology such as "brain death" without specifying the mechanism (e.g., "cerebral edema with transtentorial herniation secondary to NORSE") 1.

Document treatment-related complications appropriately. If death occurs as a direct consequence of anesthetic agents used for seizure control (propofol, pentobarbital, midazolam), this should be noted in Part II as a contributing factor, not as the primary cause 6, 7, 8.

Time Interval Documentation

Include approximate time intervals between each condition when known, as this provides important epidemiological data 2:

• Example: "Cardiorespiratory arrest (minutes) due to New-Onset Refractory Status Epilepticus (26 days) due to Autoimmune encephalitis (unknown duration)" 3

Special Circumstances

FIRES (Febrile Infection-Related Epilepsy Syndrome)

When NORSE is preceded by febrile illness 2 weeks to 24 hours prior to onset, document as "Febrile Infection-Related Epilepsy Syndrome (FIRES)" rather than NORSE, as this represents a specific subcategory 1.

Pediatric Cases with Specific Radiographic Patterns

In young children with NORSE demonstrating diffuse cerebral restriction in diffusion (DCRD), consider documenting this radiographic finding as it may represent a novel phenotype with specific prognostic implications 9.