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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

Preoperative magnetic resonance images 2 months after discharge. T2 signal changes and contrast enhancement pattern has increased, since the last magnetic resonance imaging scan
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Figure 5: Preoperative magnetic resonance images 2 months after discharge. T2 signal changes and contrast enhancement pattern has increased, since the last magnetic resonance imaging scan

Mentions: The patient's clinical improvement did not last and approximately 1-week after discharge his short-term memory loss returned and he remained unable to store new memories. He completed his antibiotic course and outpatient follow-up visits over the next 2 months revealed no improvement in his symptomatology. Given the patient's poor quality of life and the absence of diagnosis despite exhaustive testing, the patient was readmitted for right temporal craniotomy and biopsy to establish a tissue diagnosis. Examination at that time was significant for inability to recall items moments after they were told to him. He was awake, alert and oriented to person, place, and time. No focal deficits were noted. A new preoperative MRI showed interval progression of T2 signal abnormality and enhancement of the bilateral temporal lobes with increased mass effect [Figure 5]. The pituitary gland remained stable.


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)

Preoperative magnetic resonance images 2 months after discharge. T2 signal changes and contrast enhancement pattern has increased, since the last magnetic resonance imaging scan
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 5: Preoperative magnetic resonance images 2 months after discharge. T2 signal changes and contrast enhancement pattern has increased, since the last magnetic resonance imaging scan
Mentions: The patient's clinical improvement did not last and approximately 1-week after discharge his short-term memory loss returned and he remained unable to store new memories. He completed his antibiotic course and outpatient follow-up visits over the next 2 months revealed no improvement in his symptomatology. Given the patient's poor quality of life and the absence of diagnosis despite exhaustive testing, the patient was readmitted for right temporal craniotomy and biopsy to establish a tissue diagnosis. Examination at that time was significant for inability to recall items moments after they were told to him. He was awake, alert and oriented to person, place, and time. No focal deficits were noted. A new preoperative MRI showed interval progression of T2 signal abnormality and enhancement of the bilateral temporal lobes with increased mass effect [Figure 5]. The pituitary gland remained stable.

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