What is the possible diagnosis for a patient presenting with repeated episodes of hypothermia, encephalopathy, and hypertension?

Differential Diagnosis for Repeated Hypothermia, Encephalopathy, and Hypertension

The triad of recurrent hypothermia, encephalopathy, and hypertension should prompt immediate consideration of Wernicke's encephalopathy, posterior reversible encephalopathy syndrome (PRES), or acute liver failure with hepatic encephalopathy, though none of these evidence sources directly address this specific clinical presentation.

Critical Diagnostic Approach

Based on the available evidence, this clinical triad is not specifically addressed in the provided guidelines. However, the evidence does reveal important considerations:

Temperature Dysregulation Context

• Hypothermia with encephalopathy typically occurs as a consequence rather than a cause in critical illness, with neurological dysfunction developing when core temperature drops below 30°C, manifesting as confusion, incoordination, somnolence, and eventual coma 1
• Patients become comatose around 30°C with loss of deep tendon reflexes and pupillary reflexes below 27°C 1
• Brain death cannot be diagnosed during severe hypothermia; patients must be rewarmed to 34°C before neurological assessment 1

Hypertension with Encephalopathy Patterns

• Acute hypertension frequently accompanies stroke and other neurological emergencies, though marked blood pressure changes may indicate underlying conditions such as aortic dissection or myocardial infarction requiring further diagnostic evaluation 1
• Systolic blood pressure >220 mm Hg or diastolic pressure >105 mm Hg increases risk of hemorrhagic transformation in stroke patients 1
• Hypertension with encephalopathy in the context of intracranial pathology requires careful blood pressure management, as specific targets vary widely in practice 1

Recurrent Presentation Considerations

The repeated nature of these episodes is the most diagnostically significant feature, suggesting:

• Metabolic or endocrine disorders (hypothyroidism, adrenal insufficiency, Wernicke's encephalopathy)
• Toxic exposures with intermittent exposure patterns
• Posterior reversible encephalopathy syndrome (PRES) with recurrent triggers
• Acute intermittent porphyria or other metabolic crises
• Sepsis with autonomic dysfunction causing temperature dysregulation

Essential Immediate Workup

Obtain the following without delay:

• Core temperature monitoring (esophageal, bladder, or pulmonary artery catheter preferred over rectal or axillary) 1
• Comprehensive metabolic panel including glucose, electrolytes, renal and hepatic function 1
• Thyroid function tests (TSH, free T4)
• Cortisol level and ACTH stimulation test
• Thiamine level and immediate empiric thiamine replacement
• Ammonia level if liver dysfunction suspected 1
• Toxicology screen
• Brain imaging (non-contrast CT initially, MRI if PRES suspected) 1
• Blood cultures if infection suspected 2

Management Priorities During Episodes

Temperature Management:

• Rewarm hypothermic patients gradually to avoid cardiovascular complications, as mild hypothermia (<36°C) causes increased sympathetic tone while moderate hypothermia depresses cardiac activity 1
• Target normothermia (36.0-37.5°C) once rewarmed, as temperature extremes worsen neurological outcomes 2
• Use central temperature monitoring for accuracy 2

Blood Pressure Management:

• Avoid aggressive antihypertensive therapy unless systolic BP >220 mm Hg or diastolic >105 mm Hg to prevent hemorrhagic transformation 1
• Consider underlying causes (aortic dissection, myocardial infarction) if marked BP elevation 1

Encephalopathy Management:

• Rule out confounding factors including sedatives, electrolyte disturbances, and hypothermia before prognostication 1
• If hepatic encephalopathy suspected, use nonabsorbable disaccharides (lactulose) or enteral polyethylene glycol with rifaximin as adjunctive therapy 1
• Consider hypertonic saline if intracranial hypertension develops in acute liver failure 1

Critical Pitfalls to Avoid

• Do not attribute hypothermia to environmental exposure alone without excluding metabolic, endocrine, and toxic causes in recurrent presentations
• Do not perform neurological prognostication until core temperature reaches at least 34°C 1
• Do not delay empiric thiamine administration (500 mg IV) while awaiting laboratory confirmation if Wernicke's encephalopathy is possible
• Do not assume fever indicates infection in neurological emergencies; central fever from pontine or hypothalamic injury can occur 2