Nothnagel Syndrome: A rare case of Ischemic Brain Stem Syndrome

‘Nothnagel Syndrome' is a brainstem syndrome which is often underdiagnosed by the physicians due to rarity of the disease and complex clinical presentation. We present a case of brainstem midbrain syndrome caused by ischemic stroke. The male hypertensive diabetic patient presented with sudden onset of diplopia, ptosis and gait imbalance. Right-sidedgaze palsy with nystagmus and ataxic gait were the clinical findings. Brain imaging (MRI and DWI) showed high signal intensity at upper midbrain. The patient was finally diagnosed as ischemic stroke in the brainstem called Nothnagel syndrome. Up to now, there was no case reported as Nothnagel Syndrome due to ischemic stroke. J MEDICINE JAN 2020; 21 (1) : 62-64

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.