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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: 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.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.

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

Axial magnetic resonance T2 flair image on the left and postcontrast T1 image on the right showing interval improvement in enhancement pattern with stable hyperintensity of bilateral mesial temporal lobes
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Figure 4: Axial magnetic resonance T2 flair image on the left and postcontrast T1 image on the right showing interval improvement in enhancement pattern with stable hyperintensity of bilateral mesial temporal lobes

Mentions: A repeat lumbar puncture performed 5 days later revealed slight improvement in CNS inflammation. Repeat testing through PCR, ELISA, and culture for HSV 1 and 2 remained negative. Repeat imaging showed unchanged T2 hyperintensities in bilateral temporal lobes with improvement in the enhancement pattern of the left temporal lobe [Figure 4]. The patient was discharged on IV acyclovir and a steroid taper.


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)

Axial magnetic resonance T2 flair image on the left and postcontrast T1 image on the right showing interval improvement in enhancement pattern with stable hyperintensity of bilateral mesial temporal lobes
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: Axial magnetic resonance T2 flair image on the left and postcontrast T1 image on the right showing interval improvement in enhancement pattern with stable hyperintensity of bilateral mesial temporal lobes
Mentions: A repeat lumbar puncture performed 5 days later revealed slight improvement in CNS inflammation. Repeat testing through PCR, ELISA, and culture for HSV 1 and 2 remained negative. Repeat imaging showed unchanged T2 hyperintensities in bilateral temporal lobes with improvement in the enhancement pattern of the left temporal lobe [Figure 4]. The patient was discharged on IV acyclovir and a steroid taper.

Bottom Line: 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.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.

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