Endocrine Abstracts

Introduction

Non-functioning secretory pituitary adenomas are usually characterized by the lack of associated hormone overproduction. The absence of symptoms of excess hormone production results in a delayed diagnosis: sometimes as an incidental diagnosis, sometimes due to compressive symptoms with visual involvement or neurological symptoms as headache. Howewer, hydrocephalus associating Hakim-Adams Syndrome caused by foramen of Monro obstruction secondary to pituitary adenoma is an unusual manifestation.

Case presentation

We present the case of a 64-year-old man admitted to Emergency Department for a gradual development of gait disturbance and primarily urinary incontinence for the past three months, as well as the onset of cognitive impairment (disorientation, frequent medication forgetfulness, driving disability). He had a previous medical history of hypertension and chronic daily headaches. He had recently consulted Neurology, being diagnosed of atypical parkinsonian syndrome and having started the combination of levodopa and carbidopa medication, with no apparent improvement. At physical examination he presented decreased alertness, memory loss and postural instability. His walk was characterized by short and shuffling steps on widely spaced legs and loss of balance. Head magnetic resonance (MR) revealed a 44 × 48 × 35 mm pituitary Knosp 3a macroadenoma with suprasellar extension, compressing the optic chiasma and associating a remarkable hydrocephalus, particularly of the right ventricle resulting from obstruction of the foramen of Monro. Visual field revealed paracentral scotomas. On admission, laboratory findings showed prolactin levels 9.2 ng/ml [2.6–13.1], TSH and T4L levels 0.40 mUI/l [0.380–5.330] and 0.58 ng/dl [0.54–1.24], respectively; IGF-1 levels 82.6 ng/ml [67–141] and testosterone levels < 0.10 ng/ml [1.75–7.81]. LH 0.42 UI/l [1.2–8.6] and FSH 2.93 UI/l [1.27–19.26]. Cortisol and ACTH not valuable due to treatment with dexamethasone when MR images were assessed by neurosurgery on admission to the emergency room. Sodium levels 135 mmol/l [134–145], without any clinical symptoms suggestive of diabetes insipidus. Due to COVID-19 pandemic and overcrowding hospital the patient had to be transferred to a free-Covid hospital for surgery, with pathological pattern and immunohistochemical analysis pending.

Conclusion

Hydrocephalus as a complication of pituitary adenomas is infrequent. Pituitary tumours rarely become large enough to cause an obstruction at the foramen of Monro. This case is an illustration of this unusual clinical manifestation: hydrocephalus caused by foramen of Monro obstruction associating the classical picture of Hakim-Adams Syndrome in a patient misdiagnosed with atypical parkinsonian syndrome. The considerable overlap of symptoms for atypical parkinsonian syndromes with symptomatic hydrocephalus makes clinical diagnosis challenging and may lead to delay in diagnosis.