Idiopathic Intracranial Hypertension following Levothyroxine Replacement Therapy: Systematic Review and a Case Report

Abstract

The data on the characteristics of patients with idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement are limited. Here, we report a case and systematically review published cases of idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement. The systematic review was performed as per the PRISMA guidelines. Our patient is a 46-year-old lady with hypothyroidism (thyrotropin: 319 mIU/L, free thyroxine: 0.04 ng/dl), treated with 100 μg.d of LT4 and presented a month later with headache, visual diminution, bilateral lateral rectus palsies, and papilledema. Cerebrospinal fluid (CSF) pressure was 32 cmH2O. Drainage of CSF, oral acetazolamide, and modification of LT4 dose resulted in prompt symptomatic improvement and complete reversal of IIH. In the systematic review (n = 21), the median age of patients (7 males) was 13 (IQR: 8.8- 26.5) years. The median duration of hypothyroid symptoms was 4 (n = 10, IQR: 0.44-6.25) years whereas that from initiation of LT4 replacement to the diagnosis of IIH was 2 (n = 20, IQR: 1.17-4) months. Initial median serum thyrotropin and thyroxine were 100 (n = 14, IQR: 72.5-421.6) mIU/L, and 1.13 (n = 12, IQR: 1.0-2.45) μg/dl which changed to 2.2 (n = 7; IQR: 0.23-3.40) mIU/L and 8.90 μg/dl (n = 8, IQR: 6.43-14.85 μg/dl), respectively at diagnosis of IIH after LT4 treatment with median daily LT4 doses of 0.89 (n = 8, IQR: 0.60 - 1.17) times the maximum recommended dose for age. To conclude, we report an adult woman with IIH following LT4 replacement for primary hypothyroidism, a rare entity. Pediatric age, prolonged symptom duration, and use of higher LT4 replacement dose may be associated with IIH following LT4 replacement.

Keywords: Hypothyroidism; idiopathic intracranial hypertension; papilledema; pseudotumor cerebri; thyroxine.

Figure 1

Preferred reporting items for systematic reviews and meta-analyses flow diagram for selecting of articles for systematic review

Figure 2

Fundus examination showing bilateral papilledema with splinter hemorrhages and absent spontaneous venous pulsations at diagnosis of idiopathic intracranial hypertension (a and b); complete resolution of papilledema with normal optic disc and spontaneous venous pulsations three months later (c and d)

Figure 3

T2-weighted sagittal (a) and T1-weighted coronal (b) magnetic resonance imaging of brain revealed a partial empty sella