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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

Magnetic resonance images obtained 1-month after surgery made with and without contrast show a decrease in size of the pituitary gland with no suprasellar extension or abnormal enhancement pattern
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Figure 2: Magnetic resonance images obtained 1-month after surgery made with and without contrast show a decrease in size of the pituitary gland with no suprasellar extension or abnormal enhancement pattern

Mentions: The final pathology, however, was interpreted to represent GH. Special stains for microorganisms were negative. Microscopically, cytologic preparations adenosine triphosphate showed predominantly blood with scattered lymphocytes and macrophages. Frozen (AFS) and permanent sections showed a pituitary gland with marked noncaseating granulomatous inflammation and diffused lymphocytic infiltrate. Immunostains such as CD68 confirmed the presence of histiocytes and CD3 and CD20 immunostains showed the lymphocytes to be a reactive population. Cytokeratin immunostains highlighted the pituitary gland and the intact acinar architecture in the background of inflammation. Special stains for fungal Gomori methenamine silver (GMS), bacterial (gram), and mycobacterial (Fite) organisms were negative. Cerebrospinal fluid (CSF) analysis was negative for malignancy, and immunohistochemical staining for herpes simplex virus (HSV1–2) was negative. The patient was discharged on postoperative day 1 neurologically intact and prescribed thyroid, testosterone, and pulsed high-dose oral steroid regimen. The patient's condition improved, and a subsequent MRI scan showed a decrease in size of pituitary back to a more normal configuration [Figure 2].


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)

Magnetic resonance images obtained 1-month after surgery made with and without contrast show a decrease in size of the pituitary gland with no suprasellar extension or abnormal enhancement pattern
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Magnetic resonance images obtained 1-month after surgery made with and without contrast show a decrease in size of the pituitary gland with no suprasellar extension or abnormal enhancement pattern
Mentions: The final pathology, however, was interpreted to represent GH. Special stains for microorganisms were negative. Microscopically, cytologic preparations adenosine triphosphate showed predominantly blood with scattered lymphocytes and macrophages. Frozen (AFS) and permanent sections showed a pituitary gland with marked noncaseating granulomatous inflammation and diffused lymphocytic infiltrate. Immunostains such as CD68 confirmed the presence of histiocytes and CD3 and CD20 immunostains showed the lymphocytes to be a reactive population. Cytokeratin immunostains highlighted the pituitary gland and the intact acinar architecture in the background of inflammation. Special stains for fungal Gomori methenamine silver (GMS), bacterial (gram), and mycobacterial (Fite) organisms were negative. Cerebrospinal fluid (CSF) analysis was negative for malignancy, and immunohistochemical staining for herpes simplex virus (HSV1–2) was negative. The patient was discharged on postoperative day 1 neurologically intact and prescribed thyroid, testosterone, and pulsed high-dose oral steroid regimen. The patient's condition improved, and a subsequent MRI scan showed a decrease in size of pituitary back to a more normal configuration [Figure 2].

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