- Email: [email protected]
An enhancing pituitary lesion in a young woman: A diagnostic dilemma
286
Case reports / Journal of Clinical Neuroscience 14 (2007) 286–288
pressures, coagulopathy, underlying disease, or cerebrospinal fluid drainage, have been described. However, these factors do not seem to be related to remote postcraniotomy hematomas.17 In our patient, bleeding from a torn bridging vein that was found during the second operation, shows that damage of venous structures secondary to parenchymal shift may cause contralateral subdural hematoma. During surgery we observed that the brain parenchyma remained depressed at the end of the first procedure, in contrast to cases where progressive brain swelling intraoperatively is caused by parenchymal shift, in association with abrupt brain re-expansion or decreased intracranial pressure.1 That is, the brain parenchyma compressed by the longstanding large subdural calcification had lost structural elasticity, and perhaps there was pre-existing brain atrophy. It is likely that repositioning of the head to neutral immediately after operation, especially where significant re-expansion of severely compressed brain has not occurred, caused mechanical parenchymal shift and diffuse arachnoid detachment from the cerebral parenchyma opposite to the craniotomy. The draining veins fixed to the detached arachnoid membrane consequently were injured and formed the subdural hematoma. Is this complication preventable? It is possible to avoid rapid intracranial shift by leaving the hard inner membrane in place. However, removal of only the outer membrane and calcified material will leave residual cerebral compression.5,20 We suggest that the safest approach if the brain parenchyma is compressed by a large subdural calcification and remains depressed at the end of surgery or re-expands insignificantly, is to maintain the head position as it was intraoperatively or at least restrict head rotation immediately after operation. References 1. Su TM, Shih TY, Yen HL, et al. Contralateral acute subdural hematoma occurring after evacuation of subdural hygroma: case report. J Trauma 2001;50:557–9. 2. Turgut M, Akalan N, Saglam S. A fatal acute subdural hematoma occurring after evacuation of ‘‘contralateral’’ chronic subdural hematoma. J Neurosurg Sci 1998;42:61–3.
3. Harada K, Ohtsuru K, Nakayama K, et al. Contralateral development of acute subdural hematoma following surgery for chronic subdural hematoma – case report (Tokyo). Neurol Med Chir 1992;32:969–71. 4. Afra D. Ossification of subdural hematoma. Report of two cases. J Neurosurg 1961;18:393–7. 5. Niwa J, Nakamura T, Fujishige M, et al. Removal of a large asymptomatic calcified chronic subdural hematoma. Surg Neurol 1988;30:135–9. 6. Kim JH, Bae HG, Lee KS, et al. A case of asymptomatic huge calcified chronic subdural hematoma: case report. J Korean Neurosurg Soc 1996;25:1717–22. 7. Sharma RR, Mahapatra A, Pawar SJ, et al. Symptomatic calcified subdural hematomas. Pediatr Neurosurg 1999;31:150–4. 8. Narsinghani U, Burson G, Schmidt MB, et al. Case of the month. Symptomatic chronic calcified subdural hematoma in a 5-year-old child. J Ark Med Soc 2002;98:300–3. 9. Imaizumi S, Onuma T, Kameyama M, et al. Organized chronic subdural hematoma requiring craniotomy – five case reports (Tokyo). Neurol Med Chir 2001;41:19–24. 10. McLaurin RL, McLaurin KS. Calcified subdural hematomas in childhood. J Neurosurg 1966;24:648–55. 11. Debois V, Lombaert A. Calcified chronic subdural hematoma. Surg Neurol 1980;14:455–8. 12. Watts C. The management of intracranial calcified subdural hematomas. Surg Neurol 1976;6:247–50. 13. Hirakawa T, Tanaka A, Yoshinaga S, et al. Calcified chronic subdural hematoma with intracerebral rupture forming a subcortical hematoma. A case report. Surg Neurol 1989;32:51–5. 14. Park JS, Son EI, Kim DW, et al. Calcified chronic subdural hematoma associated with intracerebral hematoma. J Korean Neurosurg Soc 2003;34:177–8. 15. Ide M, Jimbo M, Yamamoto M, et al. Asymptomatic calcified chronic subdural hematoma – report of three cases (Tokyo). Neurol Med Chir 1993;33:559–63. 16. Kalfas IH, Little JR. Postoperative hemorrhage: a survey of 4992 intracranial procedures. Neurosurgery 1988;23:343–7. 17. Brisman MH, Bederson JB, Sen CN, et al. Intracerebral hemorrhage occurring remote from the craniotomy site. Neurosurgery 1996;39:1114–21. 18. Rapana A, Lamaida E, Pizza V. Multiple postoperative intracerebral haematomas remote from the site of craniotomy. Br J Neurosurg 1998;12:364–8. 19. Haines SJ, Maroon JC, Jannetta PJ. Supratentorial intracerebral hemorrhage following posterior fossa surgery. J Neurosurg 1978;49: 881–6. 20. Fukazawa K, Sakakura M, Niwa S, et al. A case of organized chronic subdural hematoma showing an early recurrence after craniotomy. No Shinkei Geka 2005;33:389–94.
doi:10.1016/j.jocn.2005.11.016
An enhancing pituitary lesion in a young woman: A diagnostic dilemma Wasiq Thiryayi a
a,*
, Marcia Hugh Donaldson b, David Border c, Atul Tyagi
Department of Neurosurgery, Hope Hospital, 65 Vancouver Quay, Salford Quays Salford, Manchester, Lancashire M50 3TU, UK b Department of Neurosurgery, Leeds General Infirmary, Leeds, UK c Department of Renal medicine, Bradford Royal Infirmary, Bradford d Department of Neurosurgery, Leeds General Infirmary, Leeds, UK Received 14 October 2005; accepted 3 December 2005
*
d
Corresponding author. Tel./fax: +44 1618777 633. E-mail address: [email protected] (W. Thiryayi).
Case reports / Journal of Clinical Neuroscience 14 (2007) 286–288
287
Abstract Wegener’s granulomatosis is a systemic small-vessel vasculitis predominantly involving the upper airways, lungs and kidneys. Central nervous system involvement is known to occur in 10% of cases, manifesting as diffuse meningeal involvement and cerebal granulomatous lesions, but isolated involvement of the pituitary is extremely rare. We describe a case of isolated pituitary involvement with abscess formation secondary to Wegener’s granulomatosis in a 21-year-old woman. To the best of our knowledge this is the first reported case of pituitary abscess formation secondary to Wegner’s granulomatosis. 2006 Elsevier Ltd. All rights reserved. Keywords: Pituitary adenoma; Pituitary abscess; Granulocytic hypophysitis; Wegener’s granulomatosis
1. Clinical background A 21-year-old Asian woman, previously in excellent health, was urgently referred to our centre with a 3-week history of intense retro-orbital headaches with nausea and low grade intermittent fever. She also had episodes of bitemporal blurring and double vision. She admitted to having been exposed, as a child, to a family member with pulmonary tuberculosis (TB). She had no rhinorrhea or any other upper respiratory or chest symptoms. A neurological examination was unremarkable in that she had normal visual acuity and normal visual fields on confrontation and no focal neurological deficits. She had no focal upper or lower respiratory tract findings. An initial noncontrast CT scan of the head was unremarkable and so was a chest Xray. An LP demonstrated raised white cells with increased neutrophils and lymphocytes with low glucose levels. A provisional diagnosis of bacterial meningitis was made and she was started empirically on intravenous cefotaxime pending microbiology results. Four days later she deteriorated with worsening headaches and persistent visual field defects. An MR scan of the brain with contrast was performed revealing a large sellar mass with central hypo-intensity and peripheral ring-form hyperintensity, extending into the
supra-sellar cistern (Figs. 1–3). In addition, there was a small rim of enhancing sphenoidal sinus mucosa under the sellar floor with the appearance of a non-enhancing lesion in the anterior sinus cavity. Pituitary function tests conducted at the time revealed no biochemical evidence of pituitary dysfunction, with normal prolactin and anterior pituitary hormone levels. She underwent an urgent transphenoidal exploration for drainage of the abscess. At surgery, inspissated fluid was noted in the cavity of the sphenoid sinus and a sellar lesion with friable lining was seen with biopsies of the lesion and its contents revealing partly granulomatous necrotising inflammatory tissue. However, no organisms or acid fast bacilli were seen or grown on serial cultures of the tissue obtained and PCR analysis on the sample revealed no evidence of TB. She developed transient diabetes insipidus postoperatively, and was started on hormone replacement therapy for decreased gonadotropins. Serum levels of all other pituitary hormones were normal and as the random serum cortisol levels tested on days 3 and 4 after the procedure were above 600 nmol/L, the short synacthen test was not performed. CSF virology and all fungal cultures from specimens taken at the time of surgery were negative as were the serological tests for mycoplasma, Q-fever, psittacosis, brucella, and TB. She was discharged
Figs. 1–3. (left to right) Axial, coronal and sagittal T2-weighted MRI images showing sellar ring enhancing lesion with suprasellar extension.
288
Case reports / Journal of Clinical Neuroscience 14 (2007) 286–288
home following a normal glucagon stimulation test with improving headaches and no visual symptoms. Ten days later, she returned with intermittent fevers and raised inflammatory markers (erythrocyte sedimentation rate [ESR] and C-reactive protein). Additionally she developed increasing fatigue, lethargy and generalised arthralgias with loss of weight and appetite. Neurological examination was normal and there was no evidence of a central nervous system infection or residual abscess. Further serological work-up revealed rising anti-neutrophil cytoplasmic antibody with cytoplasmic distribution (cANCA) titres to levels greater than 1/80, diagnostic of Wegener’s granulomatosis. Again a repeat chest X-ray revealed no evidence of pulmonary involvement and there was no suggestion of renal or sinus involvement, with a normal urine analysis and serum renal profile. She was started on pulse steroids (prednisolone) with cyclophosphamide. She responded to immunosuppressive therapy with a sustained improvement of her symptoms and a gradual decrease of the c-ANCA titres and was discharged soon thereafter. No follow-up imaging was performed and she has remained well at subsequent outpatient reviews, requiring no further gonadotropin supplements as she had no evidence of residual pituitary dysfunction on subsequent biochemical tests. 2. Discussion Wegener’s granulomatosis is a systemic necrotising granulomatous vasculitis predominantly involving the upper airways, lungs and kidneys. In addition to the classic triad of upper and lower respiratory tract and kidney disease, virtually any organ can be involved with vasculitis, granuloma, or both. Central nervous system involvement is known to occur in 10% of cases,1 manifesting as diffuse meningeal involvement and cerebral granulomatous lesions, but isolated involvement of the pituitary is extremely rare. The disease can present at any age, and approximately 15% of patients are less than 19 years of age at first presentation, with the mean age of onset being the 4th and 5th decades.2 Neurological presentations may include cranial neuritis, mononeuritis multiplex, or rarely cerebral vasculitis and granuloma formation involving the brain parenchyma or the meninges.3 Rarer still is the involvement of the pituitary gland alone causing central diabetes insipidus, without respiratory or renal involvement.4–6 Central diabetes insipidus can be inaugural in 46% of cases with pituitary involvement.4 Isolated pituitary Wegener’s granulomatosis has been reported previously, but in cases described, there was evidence of pituitary or other organ system dysfunction triggering the investigations.1 Wegener’s granulomatosis is unusual in those under 20 years of age, with 85% of cases occurring in those over the age of 19.2 In a review of 50 cases of Wegener’s granudoi:10.1016/j.jocn.2005.12.005
lomatosis, Halstead et al. revealed that 10 (20%) of these patients were under 25, with ages ranging from 13–23 years.7 Closer examination of this younger group revealed striking differences in their presenting symptoms and organ involvement when compared to the older group of patients. Two of the three patients with intracranial involvement were in the younger age group, with all the younger patients having manifestations of the disease in the head and neck. The majority of patients reported in the literature on Wegener’s granulomatosis with pituitary involvement, have had other evidence of activity of the disease, including sinusitis, lung nodules, nephropathy, cutaneous vasculitis, and arthritis. The absence of these in our patient at the time of initial presentation is distinctly unusual. Characteristic laboratory findings include a markedly elevated ESR, mild anaemia, leucocytosis and elevated cANCA levels in the acute phase. In typical Wegener’s granulomatosis with granulomatous vasculitis of the respiratory tract and glomerulonephritis, the specificity of a positive c-ANCA is very high, with approximately 90% of patients having a positive c-ANCA.3 This report highlights the need to consider the diagnosis of Wegener’s granulomatosis in the younger patient presenting with nonspecific systemic signs and focal inflammatory lesions of the central nervous system, as it may be the primary site of localization of the disease process, without any other organ system involvement. The diagnosis, if made early, and the treatment, if started appropriately, may help prevent spread to other sites, when the disease becomes more aggressive and the morbidity more significant. References 1. Ossi E, Rossanese A, Casagrande F, et al. Predictive value of ANCA in atypical primary brain localization of Wegener’s granulomatosis. Sarcoidosis Vasc Diffuse Lung Dis 2002;19:78–80. 2. de la Cruz A, Gonzalez Diaz R, Lopez Lara ND. Wegener’s granulomatosis. Report of a paediatric case and review of the literature. Rev Alerg Mex 2003;50:71–5. 3. Fauci AS. The vasculitis syndromes. In: Fauci AS, Braunwald E, Isselbacher KJ, et al., editors. Harrison’s Principles of Internal Medicine. New York: McGraw Hill; 2001. p. 1960–3. 4. Berthier S, Gil H, Bonneville JF, et al. Wegener’s disease and central diabetes insipidus. Ann Endocrinol (Paris) 2000;61: 531–7. 5. Roberts GA, Eren E, Sinclair H, et al. Two cases of Wegener’s granulomatosis involving the pituitary. Clin Endocrinol (Oxf) 1995;42:323–8. 6. Goyal M, Kucharczyk W, Keystone E. Granulomatous hypophysitis due to Wegener’s granulomatosis. AJNR Am J Neuroradiol 2000;21:1466–9. 7. Halstead LA, Karmody CS, Wolff SM. Presentation of Wegener’s granulomatosis in young patients. Otolaryngol Head Neck Surg 1986;94:368–71.
Case reports / Journal of Clinical Neuroscience 14 (2007) 286–288
pressures, coagulopathy, underlying disease, or cerebrospinal fluid drainage, have been described. However, these factors do not seem to be related to remote postcraniotomy hematomas.17 In our patient, bleeding from a torn bridging vein that was found during the second operation, shows that damage of venous structures secondary to parenchymal shift may cause contralateral subdural hematoma. During surgery we observed that the brain parenchyma remained depressed at the end of the first procedure, in contrast to cases where progressive brain swelling intraoperatively is caused by parenchymal shift, in association with abrupt brain re-expansion or decreased intracranial pressure.1 That is, the brain parenchyma compressed by the longstanding large subdural calcification had lost structural elasticity, and perhaps there was pre-existing brain atrophy. It is likely that repositioning of the head to neutral immediately after operation, especially where significant re-expansion of severely compressed brain has not occurred, caused mechanical parenchymal shift and diffuse arachnoid detachment from the cerebral parenchyma opposite to the craniotomy. The draining veins fixed to the detached arachnoid membrane consequently were injured and formed the subdural hematoma. Is this complication preventable? It is possible to avoid rapid intracranial shift by leaving the hard inner membrane in place. However, removal of only the outer membrane and calcified material will leave residual cerebral compression.5,20 We suggest that the safest approach if the brain parenchyma is compressed by a large subdural calcification and remains depressed at the end of surgery or re-expands insignificantly, is to maintain the head position as it was intraoperatively or at least restrict head rotation immediately after operation. References 1. Su TM, Shih TY, Yen HL, et al. Contralateral acute subdural hematoma occurring after evacuation of subdural hygroma: case report. J Trauma 2001;50:557–9. 2. Turgut M, Akalan N, Saglam S. A fatal acute subdural hematoma occurring after evacuation of ‘‘contralateral’’ chronic subdural hematoma. J Neurosurg Sci 1998;42:61–3.
3. Harada K, Ohtsuru K, Nakayama K, et al. Contralateral development of acute subdural hematoma following surgery for chronic subdural hematoma – case report (Tokyo). Neurol Med Chir 1992;32:969–71. 4. Afra D. Ossification of subdural hematoma. Report of two cases. J Neurosurg 1961;18:393–7. 5. Niwa J, Nakamura T, Fujishige M, et al. Removal of a large asymptomatic calcified chronic subdural hematoma. Surg Neurol 1988;30:135–9. 6. Kim JH, Bae HG, Lee KS, et al. A case of asymptomatic huge calcified chronic subdural hematoma: case report. J Korean Neurosurg Soc 1996;25:1717–22. 7. Sharma RR, Mahapatra A, Pawar SJ, et al. Symptomatic calcified subdural hematomas. Pediatr Neurosurg 1999;31:150–4. 8. Narsinghani U, Burson G, Schmidt MB, et al. Case of the month. Symptomatic chronic calcified subdural hematoma in a 5-year-old child. J Ark Med Soc 2002;98:300–3. 9. Imaizumi S, Onuma T, Kameyama M, et al. Organized chronic subdural hematoma requiring craniotomy – five case reports (Tokyo). Neurol Med Chir 2001;41:19–24. 10. McLaurin RL, McLaurin KS. Calcified subdural hematomas in childhood. J Neurosurg 1966;24:648–55. 11. Debois V, Lombaert A. Calcified chronic subdural hematoma. Surg Neurol 1980;14:455–8. 12. Watts C. The management of intracranial calcified subdural hematomas. Surg Neurol 1976;6:247–50. 13. Hirakawa T, Tanaka A, Yoshinaga S, et al. Calcified chronic subdural hematoma with intracerebral rupture forming a subcortical hematoma. A case report. Surg Neurol 1989;32:51–5. 14. Park JS, Son EI, Kim DW, et al. Calcified chronic subdural hematoma associated with intracerebral hematoma. J Korean Neurosurg Soc 2003;34:177–8. 15. Ide M, Jimbo M, Yamamoto M, et al. Asymptomatic calcified chronic subdural hematoma – report of three cases (Tokyo). Neurol Med Chir 1993;33:559–63. 16. Kalfas IH, Little JR. Postoperative hemorrhage: a survey of 4992 intracranial procedures. Neurosurgery 1988;23:343–7. 17. Brisman MH, Bederson JB, Sen CN, et al. Intracerebral hemorrhage occurring remote from the craniotomy site. Neurosurgery 1996;39:1114–21. 18. Rapana A, Lamaida E, Pizza V. Multiple postoperative intracerebral haematomas remote from the site of craniotomy. Br J Neurosurg 1998;12:364–8. 19. Haines SJ, Maroon JC, Jannetta PJ. Supratentorial intracerebral hemorrhage following posterior fossa surgery. J Neurosurg 1978;49: 881–6. 20. Fukazawa K, Sakakura M, Niwa S, et al. A case of organized chronic subdural hematoma showing an early recurrence after craniotomy. No Shinkei Geka 2005;33:389–94.
doi:10.1016/j.jocn.2005.11.016
An enhancing pituitary lesion in a young woman: A diagnostic dilemma Wasiq Thiryayi a
a,*
, Marcia Hugh Donaldson b, David Border c, Atul Tyagi
Department of Neurosurgery, Hope Hospital, 65 Vancouver Quay, Salford Quays Salford, Manchester, Lancashire M50 3TU, UK b Department of Neurosurgery, Leeds General Infirmary, Leeds, UK c Department of Renal medicine, Bradford Royal Infirmary, Bradford d Department of Neurosurgery, Leeds General Infirmary, Leeds, UK Received 14 October 2005; accepted 3 December 2005
*
d
Corresponding author. Tel./fax: +44 1618777 633. E-mail address: [email protected] (W. Thiryayi).
Case reports / Journal of Clinical Neuroscience 14 (2007) 286–288
287
Abstract Wegener’s granulomatosis is a systemic small-vessel vasculitis predominantly involving the upper airways, lungs and kidneys. Central nervous system involvement is known to occur in 10% of cases, manifesting as diffuse meningeal involvement and cerebal granulomatous lesions, but isolated involvement of the pituitary is extremely rare. We describe a case of isolated pituitary involvement with abscess formation secondary to Wegener’s granulomatosis in a 21-year-old woman. To the best of our knowledge this is the first reported case of pituitary abscess formation secondary to Wegner’s granulomatosis. 2006 Elsevier Ltd. All rights reserved. Keywords: Pituitary adenoma; Pituitary abscess; Granulocytic hypophysitis; Wegener’s granulomatosis
1. Clinical background A 21-year-old Asian woman, previously in excellent health, was urgently referred to our centre with a 3-week history of intense retro-orbital headaches with nausea and low grade intermittent fever. She also had episodes of bitemporal blurring and double vision. She admitted to having been exposed, as a child, to a family member with pulmonary tuberculosis (TB). She had no rhinorrhea or any other upper respiratory or chest symptoms. A neurological examination was unremarkable in that she had normal visual acuity and normal visual fields on confrontation and no focal neurological deficits. She had no focal upper or lower respiratory tract findings. An initial noncontrast CT scan of the head was unremarkable and so was a chest Xray. An LP demonstrated raised white cells with increased neutrophils and lymphocytes with low glucose levels. A provisional diagnosis of bacterial meningitis was made and she was started empirically on intravenous cefotaxime pending microbiology results. Four days later she deteriorated with worsening headaches and persistent visual field defects. An MR scan of the brain with contrast was performed revealing a large sellar mass with central hypo-intensity and peripheral ring-form hyperintensity, extending into the
supra-sellar cistern (Figs. 1–3). In addition, there was a small rim of enhancing sphenoidal sinus mucosa under the sellar floor with the appearance of a non-enhancing lesion in the anterior sinus cavity. Pituitary function tests conducted at the time revealed no biochemical evidence of pituitary dysfunction, with normal prolactin and anterior pituitary hormone levels. She underwent an urgent transphenoidal exploration for drainage of the abscess. At surgery, inspissated fluid was noted in the cavity of the sphenoid sinus and a sellar lesion with friable lining was seen with biopsies of the lesion and its contents revealing partly granulomatous necrotising inflammatory tissue. However, no organisms or acid fast bacilli were seen or grown on serial cultures of the tissue obtained and PCR analysis on the sample revealed no evidence of TB. She developed transient diabetes insipidus postoperatively, and was started on hormone replacement therapy for decreased gonadotropins. Serum levels of all other pituitary hormones were normal and as the random serum cortisol levels tested on days 3 and 4 after the procedure were above 600 nmol/L, the short synacthen test was not performed. CSF virology and all fungal cultures from specimens taken at the time of surgery were negative as were the serological tests for mycoplasma, Q-fever, psittacosis, brucella, and TB. She was discharged
Figs. 1–3. (left to right) Axial, coronal and sagittal T2-weighted MRI images showing sellar ring enhancing lesion with suprasellar extension.
288
Case reports / Journal of Clinical Neuroscience 14 (2007) 286–288
home following a normal glucagon stimulation test with improving headaches and no visual symptoms. Ten days later, she returned with intermittent fevers and raised inflammatory markers (erythrocyte sedimentation rate [ESR] and C-reactive protein). Additionally she developed increasing fatigue, lethargy and generalised arthralgias with loss of weight and appetite. Neurological examination was normal and there was no evidence of a central nervous system infection or residual abscess. Further serological work-up revealed rising anti-neutrophil cytoplasmic antibody with cytoplasmic distribution (cANCA) titres to levels greater than 1/80, diagnostic of Wegener’s granulomatosis. Again a repeat chest X-ray revealed no evidence of pulmonary involvement and there was no suggestion of renal or sinus involvement, with a normal urine analysis and serum renal profile. She was started on pulse steroids (prednisolone) with cyclophosphamide. She responded to immunosuppressive therapy with a sustained improvement of her symptoms and a gradual decrease of the c-ANCA titres and was discharged soon thereafter. No follow-up imaging was performed and she has remained well at subsequent outpatient reviews, requiring no further gonadotropin supplements as she had no evidence of residual pituitary dysfunction on subsequent biochemical tests. 2. Discussion Wegener’s granulomatosis is a systemic necrotising granulomatous vasculitis predominantly involving the upper airways, lungs and kidneys. In addition to the classic triad of upper and lower respiratory tract and kidney disease, virtually any organ can be involved with vasculitis, granuloma, or both. Central nervous system involvement is known to occur in 10% of cases,1 manifesting as diffuse meningeal involvement and cerebral granulomatous lesions, but isolated involvement of the pituitary is extremely rare. The disease can present at any age, and approximately 15% of patients are less than 19 years of age at first presentation, with the mean age of onset being the 4th and 5th decades.2 Neurological presentations may include cranial neuritis, mononeuritis multiplex, or rarely cerebral vasculitis and granuloma formation involving the brain parenchyma or the meninges.3 Rarer still is the involvement of the pituitary gland alone causing central diabetes insipidus, without respiratory or renal involvement.4–6 Central diabetes insipidus can be inaugural in 46% of cases with pituitary involvement.4 Isolated pituitary Wegener’s granulomatosis has been reported previously, but in cases described, there was evidence of pituitary or other organ system dysfunction triggering the investigations.1 Wegener’s granulomatosis is unusual in those under 20 years of age, with 85% of cases occurring in those over the age of 19.2 In a review of 50 cases of Wegener’s granudoi:10.1016/j.jocn.2005.12.005
lomatosis, Halstead et al. revealed that 10 (20%) of these patients were under 25, with ages ranging from 13–23 years.7 Closer examination of this younger group revealed striking differences in their presenting symptoms and organ involvement when compared to the older group of patients. Two of the three patients with intracranial involvement were in the younger age group, with all the younger patients having manifestations of the disease in the head and neck. The majority of patients reported in the literature on Wegener’s granulomatosis with pituitary involvement, have had other evidence of activity of the disease, including sinusitis, lung nodules, nephropathy, cutaneous vasculitis, and arthritis. The absence of these in our patient at the time of initial presentation is distinctly unusual. Characteristic laboratory findings include a markedly elevated ESR, mild anaemia, leucocytosis and elevated cANCA levels in the acute phase. In typical Wegener’s granulomatosis with granulomatous vasculitis of the respiratory tract and glomerulonephritis, the specificity of a positive c-ANCA is very high, with approximately 90% of patients having a positive c-ANCA.3 This report highlights the need to consider the diagnosis of Wegener’s granulomatosis in the younger patient presenting with nonspecific systemic signs and focal inflammatory lesions of the central nervous system, as it may be the primary site of localization of the disease process, without any other organ system involvement. The diagnosis, if made early, and the treatment, if started appropriately, may help prevent spread to other sites, when the disease becomes more aggressive and the morbidity more significant. References 1. Ossi E, Rossanese A, Casagrande F, et al. Predictive value of ANCA in atypical primary brain localization of Wegener’s granulomatosis. Sarcoidosis Vasc Diffuse Lung Dis 2002;19:78–80. 2. de la Cruz A, Gonzalez Diaz R, Lopez Lara ND. Wegener’s granulomatosis. Report of a paediatric case and review of the literature. Rev Alerg Mex 2003;50:71–5. 3. Fauci AS. The vasculitis syndromes. In: Fauci AS, Braunwald E, Isselbacher KJ, et al., editors. Harrison’s Principles of Internal Medicine. New York: McGraw Hill; 2001. p. 1960–3. 4. Berthier S, Gil H, Bonneville JF, et al. Wegener’s disease and central diabetes insipidus. Ann Endocrinol (Paris) 2000;61: 531–7. 5. Roberts GA, Eren E, Sinclair H, et al. Two cases of Wegener’s granulomatosis involving the pituitary. Clin Endocrinol (Oxf) 1995;42:323–8. 6. Goyal M, Kucharczyk W, Keystone E. Granulomatous hypophysitis due to Wegener’s granulomatosis. AJNR Am J Neuroradiol 2000;21:1466–9. 7. Halstead LA, Karmody CS, Wolff SM. Presentation of Wegener’s granulomatosis in young patients. Otolaryngol Head Neck Surg 1986;94:368–71.