Encephalopathy, Chiasmal Compression, Ophthalmoplegia, and Diabetes Insipidus in Pituitary Apoplexy

Abstract

Pituitary apoplexy with haemorrhage is a potentially life-threatening condition, and a rare cause of third nerve palsies. The range of vision loss and ophthalmoplegia seen in cases of apoplexy reflects the variability of cranial structures compressed by mass effect. The pathophysiology of extraocular muscle limitation and facial paraesthesia occurs with compression of the cavernous sinus, which contains cranial nerves III, IV, VI, and the ophthalmic branch of V. Blood supply to adjacent structures may be also compromised, causing additional loss of function. This case report of a patient with diabetes insipidus and a third nerve palsy illustrates the anatomic basis of the presenting signs of pituitary apoplexy, and the necessity for prompt neuroimaging if it is suspected. Keywords: Central diabetes insipidus, pituitary apoplexy, pupil-involving third nerve palsy CASE REPORTA 50-year-old African American male with a history of hypertension presented with a 4-day history of progressive, painful vision loss in the right eye. The vision loss was described by the patient as ''closing in superiorly and inferiorly'' and progressed to no light perception in the right eye. The patient also noticed a droopy right eyelid and an outward drift of the right eye. The corresponding vision in the left eye had loss of the temporal visual field. Systemically, the patient complained of a rightsided headache, nausea, vomiting, diarrhoea, extreme thirst, and polyuria. His wife described a personality change over the preceding week. The patient denied any recent trauma or previous episodes of vision loss.On initial examination, visual acuity was no light perception in the right eye and 20/200 in the left eye. Both pupils were equal in size, but a relative afferent pupillary defect in the right eye was noted. External examination showed a ptosis and exotropia of the right eye. Confrontational visual field testing of the left eye showed complete temporal hemianopsia. The remainder of the ophthalmic examination was within normal limits. Specifically, optic nerve examination showed healthy nerves bilaterally without pallor or papilloedema. The constellation of symptoms was suspicious for an intracranial process, and a magnetic resonance imaging (MRI) was ordered. Imaging showed a reduced ventricular size and a 2.7 Â 1.7 Â 1.5 cm pituitary adenoma displacing the optic chiasm superiorly on the right with partial extension to the left side. Figure 1 A magnetic resonance angioscopy (MRA) was performed to further characterize the lesion prior to neurosurgical intervention, and it showed a left side deviation of the pituitary stalk with apoplexy and haemorrhage measuring 1.9 Â 1.9 Â 1.2 cm. The MRA also revealed 50% compression of the right internal carotid artery due to mass effect and compression of the right cavernous sinus.

Pituitary Apoplexy in Patients with Pituitary Neuroendocrine Tumors (PitNET)

“…In accordance with medical literature, we found a male predominance (male-to-female ratio of 1.76). The mean age of patients was 50.52 years, the youngest was 9, and the oldest was 85 years [ 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , ...…”

“…Other common symptoms include nausea and vomiting, either with a neurological or endocrine component (acute secondary adrenal insufficiency) [ 25 , 29 , 30 , 33 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 81 , …”

“…The most frequent cranial nerve affected was the oculomotor nerve, but rarely, patients also presented with abducens [ 25 , 44 , 77 , 81 , 84 , 85 , 91 , 98 , 113 , 120 , 121 , 123 , 124 , 128 , 142 , 168 , 174 , 182 , 192 , 207 ] and trochlear [ 25 , 63 , 81 , 84 , 102 , 121 , 123 , 124 , 149 , 168 , 182 , 209 ] nerves palsies. Optic chiasm compression was specified in 18 articles [ 32 , 33 , 46 , 55 , 62 , 63 , 65 , 69 , 80 , 123 , 125 , 169 , 177 , 185 , 186 , 191 , 192 , …”

“…Conservative management [ 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , …”

“…A large number of patients required hormonal replacement due to PA-associated hypopituitarism manifested as hypocortisolism which is the most important aspect in terms of an immediate life-threatening approach [ 25 , 29 , 30 , 31 , 56 , 65 , 66 , 75 , 80 , 90 , 92 , 123 , 128 , 144 , 163 , 168 , 169 , 208 ], central hypothyroidism [ 25 , 56 , 65 , 66 , 72 , 75 , 101 , 109 , 123 , 144 , 163 , 168 , 169 , 180 , 184 , 200 ], and hypogonadotropic hypogonadism [ 25 , 56 , 65 , 66 , 72 , 109 , 128 , 168 , 169 , 180 , 198 ]. GH deficiency was also reported [ 109 , 128 , 1...…”