What to consider in a young patient with neurological deficit following 2 weeks of headache?

Evaluation of Young Patient with Neurological Deficit Following 2 Weeks of Headache

In a young patient presenting with neurological deficits after 2 weeks of headache, immediately obtain non-contrast head CT followed by lumbar puncture if CT is negative, as this presentation mandates urgent exclusion of aneurysmal subarachnoid hemorrhage (aSAH), which carries severe morbidity and mortality risk if missed. 1

Immediate Diagnostic Workup

Primary Concern: Aneurysmal Subarachnoid Hemorrhage

• The presence of a new neurological deficit with headache is a Class I indication for non-contrast head CT followed by lumbar puncture if CT is negative, regardless of time from symptom onset 1
• The 2-week duration raises concern for a sentinel or warning headache (occurs in 10-43% of aSAH cases) that may precede catastrophic rupture 1
• Perform lumbar puncture >6 hours from most recent symptom onset to evaluate for xanthochromia using spectrophotometric analysis (sensitivity 100%, specificity 95.2%) 1
• If CT shows subarachnoid hemorrhage and high concern for aneurysmal source, digital subtraction angiography (DSA) is indicated to identify the aneurysm 1

Critical Red Flags Present in This Case

The combination of prolonged headache (2 weeks) with new neurological deficit triggers multiple red flag criteria:

• Progressive headache worsening over time suggests space-occupying lesion 2, 3
• New neurological deficit significantly increases likelihood of intracranial pathology 2
• The subacute presentation (2 weeks) distinguishes this from typical migraine with aura, which has reversible deficits lasting 5-60 minutes 4

Differential Diagnosis to Consider

After Excluding Life-Threatening Causes

If initial CT and LP are negative for SAH, obtain MRI brain with and without contrast as the preferred next imaging modality 2, 3:

• Brain tumor or other mass lesion: Progressive headache over 2 weeks with neurological deficit is classic presentation 2, 3
• Cerebral venous sinus thrombosis: Can present with subacute headache and focal deficits; requires MR venography
• Stroke or TIA: Though less common in young patients, must be excluded with MRI/MRA 1
• Cerebral arteritis or vasculitis: Can cause subacute headache with deficits; may require conventional angiography if MRI/MRA abnormal 1

Benign Mimics (Diagnoses of Exclusion)

Headache with Neurological Deficits and CSF Lymphocytosis (HaNDL) syndrome should only be considered after excluding dangerous causes 5, 6:

• Characterized by moderate-to-severe headache with focal neurological deficits and CSF lymphocytosis (mean opening pressure 240.5 mmH₂O, elevated protein in 63.4%) 5
• Most common in young adults (mean age 28.8 years) with slight male predominance 5
• Typical symptoms include unilateral severe throbbing headache with hemi-paresthesia (60%) and hemiparesis (54.8%) 5
• Brain MRI is mostly normal (78% of cases), though 20% show non-specific white matter lesions or meningeal enhancement 5, 6
• Episodes are self-limiting but can recur in 30% of patients 5
• Caution: Cerebral angiography may precipitate neurological symptoms in HaNDL patients and should be avoided unless absolutely necessary 7

Algorithmic Approach

Step 1: Emergency Evaluation (First Hour)

• Obtain non-contrast head CT immediately 1
• Perform focused neurological examination documenting specific deficits 3
• If CT positive for SAH → proceed to CTA or DSA for aneurysm identification 1

Step 2: If CT Negative (Hours 2-6)

• Perform lumbar puncture >6 hours from symptom onset 1
• Send CSF for: cell count with differential, protein, glucose, xanthochromia (spectrophotometry), Gram stain, culture 1, 5
• Measure opening pressure 5

Step 3: If LP Shows Lymphocytosis Without Xanthochromia

• Obtain MRI brain with and without contrast 2, 3
• Consider MR venography to exclude venous thrombosis
• Send additional CSF studies: oligoclonal bands, viral PCR panel, cytology 5
• Do not diagnose HaNDL until infectious, autoimmune, and neoplastic causes are excluded 5, 8

Step 4: If All Imaging and Initial Workup Negative

• Check ESR and CRP (exclude vasculitis/arteritis) 3
• Obtain EEG (may show diffuse or focal slowing in HaNDL) 5, 9
• Consider conventional angiography only if high suspicion for vasculitis and MRA non-diagnostic 1
• Repeat CSF analysis in 1-2 weeks if diagnosis remains uncertain 9

Critical Pitfalls to Avoid

• Do not assume migraine with aura in a patient with deficits lasting days to weeks; typical migraine aura resolves within 60 minutes 4
• Do not skip lumbar puncture if CT is negative; CT sensitivity for SAH decreases significantly after 6 hours, and LP is essential 1
• Do not perform LP before CT in a patient with neurological deficits, as mass effect must be excluded first
• Do not diagnose HaNDL without CSF analysis showing lymphocytosis; this is a required diagnostic criterion 5, 6
• Do not rely on normal MRI to exclude serious pathology; early tumors, vasculitis, and HaNDL can all have normal initial MRI 5, 6

Management Considerations

If Modifiable Conditions Identified

Identify and treat reversible causes early, as outcomes are significantly more favorable 1:

• Seizures, hydrocephalus, electrolyte abnormalities (especially hyponatremia), status epilepticus require immediate correction 1

If HaNDL Diagnosed

• Antivirals were most commonly used in acute phase (23.6% of cases), though evidence is limited 5
• Flunarizine most common preventive agent (3.2% of cases) 5
• Propranolol has been used successfully for prevention 9
• Reassure patient of benign, self-limiting nature with expected resolution over weeks to months 5, 8

Neurology Referral

Refer to neurology immediately for 3:

• Atypical features despite negative initial workup
• Diagnostic uncertainty
• Progressive or worsening symptoms
• Need for advanced diagnostic procedures (conventional angiography, brain biopsy)