Dorsal midbrain syndrome secondary to pineal gland tumours: case series and review on manifestations, management and outcome

Abstract Background Pineal apoplexy is a rare condition, with unknown incidence and clinical significance. To elucidate this clinical condition, we analyzed our own case series and performed a review of the literature. Methods We enrolled all patients with a hemorrhagic pineal apoplexy who were referred to our department between January 2000 and January 2020. Hemorrhagic pineal apoplexy was defined as the presence of fluid–fluid levels inside the pineal cyst (PC) on an axial or sagittal magnetic resonance scan. In one patient, after PC apoplexy, we performed a circadian melatonin sampling from peripheral blood to determine the function of the pineal gland. The PubMed database was searched for publications using the terms “pineal” and “apoplexy.” Results Eight patients were enrolled, of which three patients underwent surgical treatment and five patients were managed conservatively. One patient was tested for circadian melatonin secretion. Results confirmed melatonin secretion with preserved physiologic circadian rhythm.Our search of the literature led us to 31 studies that comprised 30 patients with apoplectic PC, 9 with apoplectic pineal tumor, and 1 with bleeding into the normal pineal gland. Most patients presented with headache, nausea, and vomiting, less frequently with acute hydrocephalus and gaze palsy. Twenty patients with a PC underwent resection or aspiration. Two patients underwent shunt placement as the only procedure and five received both shunt and surgical removal. Six patients with a PC were observed without surgical treatment. All the nine patients with a pineal tumor were operated on. In indicated cases, four patients received radiation therapy and one received chemotherapy. Conclusion Clinical significance of hemorrhagic pineal apoplexy ranges from an asymptomatic course to rapid deterioration and death. In patients with mild symptoms, observation is indicated, whereas surgical treatment is reserved for severe cases presenting with obstructive hydrocephalus and includes cerebrospinal fluid diversion, resection of apoplectic pineal lesions, or both.

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Dorsal Midbrain Syndrome

Neuro-Ophthalmology ◽

10.1093/med/9780190603953.003.0040 ◽

2019 ◽

pp. 225-230

Author(s):

Matthew J. Thurtell ◽

Robert L. Tomsak

Keyword(s):

Shunt Malfunction ◽

Upper Eyelid ◽

Convergence Insufficiency ◽

Common Causes ◽

Midbrain Syndrome ◽

Upper Eyelid Retraction ◽

Brainstem Tumors ◽

Ventricular Tumors ◽

Vertical Gaze ◽

Dorsal Midbrain Syndrome

Lesions of the dorsal midbrain produce a characteristic and highly localizing constellation of neuro-ophthalmic signs, which is known as the dorsal midbrain syndrome. In this chapter, we begin by summarizing the clinical features of the dorsal midbrain syndrome, which include supranuclear vertical gaze palsy, skew deviation, convergence insufficiency, convergence-retraction nystagmus, upper-eyelid retraction, and light-near dissociation of the pupils. We then list common causes of the dorsal midbrain syndrome, which include hydrocephalus, shunt malfunction, stroke, intrinsic brainstem tumors, and compression by extrinsic tumors, such as pineal and third ventricular tumors. Lastly, we discuss the neuro-ophthalmic features, diagnostic evaluation, and management of ventriculoperitoneal shunt malfunction.

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