Case Report Australasian Radiology (2005) 49, 127–131 pCase Report Pituitary apoplexy: An unusual cause of frontal lobe syndrome A Bhansali,1 P Dutta,1 N Khandelwal,2 A Pathak3 and RK Vashisht4 Departments of 1Endocrinology, 2Radiodiagnosis, 3Neurosurgery and 4Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India SUMMARY Pituitary apoplexy presenting as frontal lobe syndrome is rare. Interestingly, in our patient the frontal lobe infarct was as a result of intense anterior cerebral artery spasm consequent to nonaneurysmal subarachnoid haemorrhage. The mechanisms of cerebral infarct associated with pituitary apoplexy are discussed. Key words: anterior cerebral artery; cerebrovascular accident; pituitary apoplexy. INTRODUCTION CASE REPORT Pituitary apoplexy is a rare but life-threatening disorder A 30-year-old man presented with sudden onset of headache, characterized by a series of clinical events in the form of acute vomiting and complete loss of vision in the left eye and then onset of severe headache, visual impairment, ophthalmoplegia, the right eye for 10 days prior to admission. He was confused, 1 meningism and altered sensorium. These manifestations result disoriented and aggressive. However, he was obeying from acute enlargement of the pituitary gland caused commands and opening his eyes on request. On examination, by infarction and/or haemorrhage leading to compression of his blood pressure was 130/80 mmHg. He had complete loss the surrounding structures and/or extravasation of blood into of vision in both eyes with absence of perception of light, percep- 1,2 This usually occurs in an adeno- tion of rays and bilateral optic atrophy. Signs of meningeal matous gland and rarely in a normal pituitary gland. 1 Focal irritation in the form of neck stiffness and Kernig’s sign were neurological deficits in the form of hemiplegia or frontal lobe positive. He had evidence of frontal disinhibition syndrome in the syndrome in association with pituitary apoplexy have to date form of fluctuating mood, aggressive and abusive behaviour, the subarachnoid space. 3–5 These manifestations are attributed to internal disorientation with respect to time, place and person, and urinary carotid artery or anterior cerebral artery occlusion and/or incontinence. Other cranial nerves, motor and sensory systems spasm consequent on the compression of vessels by an enlarg- and systemic examinations were normal. Lumbar puncture done ing pituitary mass or vasospasm resulting from spillage of blood before admission to this hospital revealed normal cerebrospinal into the subarachnoid space. Rarely, internal carotid artery dis- fluid protein and glucose but plenty of red cells. Serum bio- section may present with pituitary apoplexy and hemiplegia chemistry showed serum sodium 130 mEq/L (normal: 128–135 because of either accelerated hypertension or atherosclerosis. 3 mEq/L), potassium 3.6 mEq/L (normal: 3.5–5.2 mEq/L), glucose We report a patient who presented with frontal lobe 3 mmol/L, T3 0.83 ng/mL (normal: 0.6–1.5 ng/mL), T4 65 ng/mL syndrome as a result of anterior cerebral artery spasm con- (normal: 60–150 ng/mL), thyrotropin 2.2 U/mL (normal: 0.5– sequent on a non-aneurysmal subarachnoid haemorrhage 4.6 U/mL), cortisol (random) 400 nmol/L (normal: >700 nmol/L (SAH) as the first presenting manifestation of haemorrhage into in stress), prolactin (PRL) 18.9 ng/mL (normal: 5–16 ng/mL), a non-functioning pituitary tumour. leutinizing hormone 2.2 mIU/mL (normal: 5–15 mIU/mL), follicular been reported. A Bhansali DM; P Dutta DM; N Khandelwal MD, DNB; A Pathak Mch; RK Vashisht MD, MRC Path. Correspondence: Dr Anil Bhansali, Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India. Email: anilbhansali_endocrine@rediffmail.com Submitted 24 February 2003; resubmitted 7 October 2003; accepted 4 July 2004. 128 A BHANSALI ET AL. stimulating hormone 2.5 mIU/mL (normal: 5–15 mIU/mL) and vascular anomaly in the region of anterior communicating testosterone 6 nmol/L (normal: 9–27 nmol/L). artery (ACOM). Digital subtraction angiography (DSA) was Plain CT of the head revealed haemorrhage into a large therefore performed. It showed typical features of a pituitary pituitary adenoma, with subarachnoid extension and a left adenoma in the form of opening up of the carotid siphon and medial frontal lobe infarct (Fig. 1). Magnetic resonance imaging elevation of the A1 segment of the anterior cerebral arteries. confirmed the same findings with a tumour size of 4 × 3 cm In addition, it revealed bilateral pencil-thin anterior cerebral with suprasellar but no parasellar extension and haemorrhage arteries suggestive of vasospasm. There was no evidence of into the tumour (Fig. 2a,b). There was a hyperintense wedge- aneurysm or any major compression at circle of Willis (Fig. 2c). shaped area in the left frontal region on MRI suggestive of The patient was managed with i.v. mannitol, dexa- infarct with subarachnoid haemorrhage in the anterior inter- methasone and haloperidol. His sensorium improved, his hemispheric fissure. However, there was a suspicion of a agitation decreased and he was discharged after 2 weeks. Re-evaluation during follow up showed that he was euthyroid and eucortisolic on replacement doses of levothyroxine and prednisolone, respectively. Repeat DSA and CT angiogram during this period showed normal calibre and filling-in of the anterior cerebral arteries and circle of Willis. There was no evidence of aneurysm or dissection (Fig. 3). He underwent trans-sphenoidal surgery uneventfully and histopathology of the tumour showed a pituitary adenoma with haemosiderin pigment-laden macrophages suggestive of old haemorrhage (Fig. 4). Immunostaining for growth hormone and PRL were negative. The patient is doing well and his vision improved in left eye to 6/6, while his right eye did not show much improvement. DISCUSSION The diagnosis of pituitary apoplexy is usually clear when there is a known tumour. However, when the presenting features are suggestive of subarachnoid haemorrhage or stroke, the underlying diagnosis may be overlooked. Bilateral optic atrophy in this setting points towards pituitary adenoma undergoing apoplexy.1 Our patient’s aggressive and violent behaviour overshadowed his other manifestations but severe headache and progressive loss of vision with bilateral optic atrophy pointed towards pituitary apoplexy with a frontal lobe infarct. The occurrence of hemiplegia or frontal lobe syndrome in patients with pituitary apoplexy is extremely unusual. 3–5 These manifestations are the result of an ischaemic insult producing cerebral infarcts. Pituitary apoplexy can cause cerebral infarction by various mechanisms.4 These include compression of an intracranial vessel by an enlarging pituitary mass, associated subarachnoid haemorrhage leading to spasm in surrounding vessels because of the irritant effect of blood, artery-to-artery embolization and hypotension.4 Rarely, ischaemia or dissection of the internal carotid artery may be the primary event leading to infarction of the pituitary adenoma. 3,5 Vasospasm as a result of the irritant effect of blood in the subarachnoid space was the most plausible explanation of frontal lobe infarct in our case at presentation. However, in view of the SAH, suspicion of a Fig. 1. (a) Plain CT shows a hyperdense mass in the suprasellar region. (b) Contrast-enhanced CT shows partial enhancement of the pituitary mass and (c) an anterior cerebral artery territory infarct on the left side. vascular anomaly in the ACOM region and previous reports of association of pituitary apoplexy with concomitant aneurysmal bleed,6 ACOM aneurysm with rupture was suspected. The demonstration of bilateral pencil-thin anterior cerebral arteries PITUITARY APOPLEXY WITH FRONTAL LOBE SYNDROME 129 Fig. 2. (a) T2-weighted coronal section of MR shows hyperintense mass in the sellar and the suprasellar region showing central hypointensity suggestive of a subacute bleed. Note hyperintensity in the left cingulate region suggestive of infarct. (b) Contrast-enhanced MR shows enhancement of the pituitary gland with central non-enhancing portion. (c) Right carotid digital subtraction angiography at the time of presentation showed spasm of supraclinoid internal carotid artery and A1 segment of the anterior cerebral artery (ACA associated with upward lifting of A1 segment). Note loop of A2 segment of the ACA. on DSA supported the hypothesis of vasospasm as an explana- re-evaluation, the DSA and CT angiogram did not reveal any tion of his clinical syndrome. This vasospasm is felt to have aneurysm or dissection, whereas normal calibre and filling-in been of sufficient magnitude to produce cerebral infarction. On of the anterior cerebral arteries and circle of Willis were seen. 130 A BHANSALI ET AL. Fig. 3. (a, b) Computed tomography angiogram on follow up showed no evidence of aneurysm or dissection. Note the loop of A2 segment of the anterior cerebral artery (ACA). (c) Follow-up right carotid digital subtraction angiography showed normal calibre of internal carotid artery and ACA. This is also counter to the common belief that non-aneurysmal 7 induce vasospasm as seen in our case. However, it depends on SAH is not usually associated with vasospasm. To our knowl- the amount of blood and the rapidity of extravasation into the edge, there is a single instance of anterior cerebral artery subarachnoid space after apoplexy. Hypothalamic damage can territory infarct in association with pituitary apoplexy reported result from compression by the expanded pituitary mass after by Majchrzak et al. In their case, there was displacement of the apoplexy. Experimental evidence suggests that destruction of carotid siphon and the A1 segment was lifted up with probable lateral hypothalamus might release some spasmogenic factors subsequent clot formation.8 and facilitate the occurrence of vasospasm. 10 Rarely, vaso- Though vasospasm in pituitary apoplexy with subarachnoid haemorrhage has been described by various authors, 4,9 the spasm can occur after pituitary surgery, and possibly humoral factors might be involved in such cases. 8 exact pathophysiology is not known. The possible explanations Early surgical intervention in patients with pituitary apoplexy are an irritant effect of subarachnoid blood, hypothalamic with stroke is debatable. It has been observed that there are damage and, occasionally following pituitary surgery, release increased chances of haemorrhage into infracted or ischemic of undefined substances from damaged cells. 10 Blood in sub- brain tissue leading to further deterioration. 11,12 We did not arachnoid space is known to be irritating to vessels and may undertake early surgery in our patient because he showed PITUITARY APOPLEXY WITH FRONTAL LOBE SYNDROME 131 2. 3. 4. 5. 6. 7. Fig. 4. Prussian blue staining showing blue-coloured haemosiderin granules (arrow) against the background of pituitary adenoma (× 440). clinical improvement with conservative management. After 8. 9. improvement in his neurological status, he underwent transsphenoidal surgery uneventfully. Histopathology of the tumour tissue showed haemosiderin pigment, confirming old haemorrhage. 10. In conclusion, our patient presented with pituitary apoplexy as the first presenting manifestation of a non-functioning pituitary tumour. 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