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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 T2 flair magnetic resonance images demonstrating hyperintensity in the bilateral mesial temporal lobes left greater than right, as well as contrast enhancement, in right mesial temporal lobe on sagittal section
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Figure 3: Axial T2 flair magnetic resonance images demonstrating hyperintensity in the bilateral mesial temporal lobes left greater than right, as well as contrast enhancement, in right mesial temporal lobe on sagittal section

Mentions: Approximately, 3 months after discharge, the patient developed fatigue, malaise, and progressive short-term memory deficits that progressed rapidly over a course of 2 weeks. He retained his remote memory and was able to recall name, birthday, and president without difficulty. There was no fever, weight loss, chills, vision changes, slurred speech, facial droop, weakness, numbness, issues with gait, or other focal deficits except for his prior hearing loss in his left ear. He was tapering his steroid medications. The patient was readmitted to the hospital for concern of HSV encephalitis. MRI showed T2 signal changes, as well as bilateral contrast enhancement, in the medial temporal lobes [Figure 3].


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 T2 flair magnetic resonance images demonstrating hyperintensity in the bilateral mesial temporal lobes left greater than right, as well as contrast enhancement, in right mesial temporal lobe on sagittal section
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Axial T2 flair magnetic resonance images demonstrating hyperintensity in the bilateral mesial temporal lobes left greater than right, as well as contrast enhancement, in right mesial temporal lobe on sagittal section
Mentions: Approximately, 3 months after discharge, the patient developed fatigue, malaise, and progressive short-term memory deficits that progressed rapidly over a course of 2 weeks. He retained his remote memory and was able to recall name, birthday, and president without difficulty. There was no fever, weight loss, chills, vision changes, slurred speech, facial droop, weakness, numbness, issues with gait, or other focal deficits except for his prior hearing loss in his left ear. He was tapering his steroid medications. The patient was readmitted to the hospital for concern of HSV encephalitis. MRI showed T2 signal changes, as well as bilateral contrast enhancement, in the medial temporal lobes [Figure 3].

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