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Primary central nervous system vasculitis preceded by granulomatous hypophysitis: Case report with a review of the literature.

Huang M, Steele WJ, Baskin DS - Surg Neurol Int (2015)

Bottom Line: Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient.We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension.He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Kenneth R. Peak Brain and Pituitary Center, Houston Methodist Neurological Institute, Houston, TX, USA.

ABSTRACT

Background: Primary central nervous system (CNS) vasculitis is an idiopathic inflammatory process that selectively affects CNS vasculature without a systemic inflammatory response, and causes luminal obstruction with resultant ischemia of recipient tissue. Its varying clinical symptoms and signs depend on the caliber of vessels involved and distribution and location of the affected structures. Granulomatous hypophysitis (GH) is an autoimmune inflammatory process typically affecting women, and usually presents with hypopituitarism, and at times, diabetes insipidus, and/or visual loss. Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient.

Case description: We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension. He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH.

Conclusion: This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH.

No MeSH data available.


Related in: MedlinePlus

Pre- and post-contrast magnetic resonance images show the pituitary to be diffusely enlarged with homogenous enhancement, measuring 1.3 cm × 2.0 cm × 1.1 cm in cranial-caudal, transverse, and anterior-posterior dimensions, respectively. The pituitary stalk is markedly thickened and intensely enhancing
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Figure 1: Pre- and post-contrast magnetic resonance images show the pituitary to be diffusely enlarged with homogenous enhancement, measuring 1.3 cm × 2.0 cm × 1.1 cm in cranial-caudal, transverse, and anterior-posterior dimensions, respectively. The pituitary stalk is markedly thickened and intensely enhancing

Mentions: A 29-year-old male initially presented to an otolaryngologist's office for evaluation of the acute onset of left-sided hearing loss. His past medical history was significant only for obesity with a body mass index 34, hypertension, and presumptive shingles on his left flank treated with valacyclovir, although no exanthema had ever appeared. He endorsed occasional alcohol but no smoking or illicit drug usage. The patient reported a 2 weeks history of headache, nausea, and vomiting was accompanied by photophobia. He had been to an emergency room, and anti-nausea and pain medications had been prescribed. While at the office, he received a local steroid injection, which provided no resolution of his symptoms. A subsequent magnetic resonance imaging (MRI) obtained at that time showed a diffusely enhancing and enlarged pituitary gland [Figure 1].


Primary central nervous system vasculitis preceded by granulomatous hypophysitis: Case report with a review of the literature.

Huang M, Steele WJ, Baskin DS - Surg Neurol Int (2015)

Pre- and post-contrast magnetic resonance images show the pituitary to be diffusely enlarged with homogenous enhancement, measuring 1.3 cm × 2.0 cm × 1.1 cm in cranial-caudal, transverse, and anterior-posterior dimensions, respectively. The pituitary stalk is markedly thickened and intensely enhancing
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4597298&req=5

Figure 1: Pre- and post-contrast magnetic resonance images show the pituitary to be diffusely enlarged with homogenous enhancement, measuring 1.3 cm × 2.0 cm × 1.1 cm in cranial-caudal, transverse, and anterior-posterior dimensions, respectively. The pituitary stalk is markedly thickened and intensely enhancing
Mentions: A 29-year-old male initially presented to an otolaryngologist's office for evaluation of the acute onset of left-sided hearing loss. His past medical history was significant only for obesity with a body mass index 34, hypertension, and presumptive shingles on his left flank treated with valacyclovir, although no exanthema had ever appeared. He endorsed occasional alcohol but no smoking or illicit drug usage. The patient reported a 2 weeks history of headache, nausea, and vomiting was accompanied by photophobia. He had been to an emergency room, and anti-nausea and pain medications had been prescribed. While at the office, he received a local steroid injection, which provided no resolution of his symptoms. A subsequent magnetic resonance imaging (MRI) obtained at that time showed a diffusely enhancing and enlarged pituitary gland [Figure 1].

Bottom Line: Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient.We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension.He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Kenneth R. Peak Brain and Pituitary Center, Houston Methodist Neurological Institute, Houston, TX, USA.

ABSTRACT

Background: Primary central nervous system (CNS) vasculitis is an idiopathic inflammatory process that selectively affects CNS vasculature without a systemic inflammatory response, and causes luminal obstruction with resultant ischemia of recipient tissue. Its varying clinical symptoms and signs depend on the caliber of vessels involved and distribution and location of the affected structures. Granulomatous hypophysitis (GH) is an autoimmune inflammatory process typically affecting women, and usually presents with hypopituitarism, and at times, diabetes insipidus, and/or visual loss. Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient.

Case description: We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension. He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH.

Conclusion: This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH.

No MeSH data available.


Related in: MedlinePlus