Neuro-Ophthalmologic Complications of Syringobulbia

Abstract

Syringobulbia is an uncommon condition, usually a late complication of syringomyelia. It has predilection for the dorsolateral region of the medulla leading to damage to vestibular nuclei and their connections, as well as to the descending sympathetic fibers. Oscillopsia, nystagmus, and Horner syndrome are frequent manifestations of syringobulbia. Oscillopsia may be a disturbing symptom for the patient, whereas Horner syndrome is usually an asymptomatic finding. MRI detection of syringomyelia has led to earlier treatment of syringomyelia and prevention of upward extension of the cavity. This probably explains why syringobulbia is less frequently encountered at present. We propose to describe the neuro-ophthalmologic symptoms and signs that may be observed in patients with syringobulbia and the mechanisms involved in their appearance.

Signs consistent with syringobulbia may be detected in dogs undergoing MRI

“…While large case studies of syringobulbia are lacking in people, reported clinical features of syringobulbia include cranial nerve abnormalities including diplopia, anisocoria, ptosis, trigeminal neuropathy, tinnitus and deafness, swallowing difficulties, dysphonia, headache, nystagmus and oscillopsia, dizziness, nausea, syncope and respiratory arrest, and autonomic dysfunction. 2,10,15,[20][21][22][23][24] The onset of clinical signs is commonly slowly progressive, and some authors have hypothesized that an acute onset of neurologic dysfunction may be prognostic for incomplete recovery following surgery. 20 Compared to our group, 75% of dogs were admitted for evaluation of a primary complaint of vestibular dysfunction, which was acute in onset in half of the cases.…”

Syringohydromyelia in association with syringobulbia and syringocephaly: case report

“…Cranial nerve involvement with CM-I abnormality with or without syringohydromyelia and syringobulbia is very common. 10,12,14,16,19,22,30,32,36 The most frequently involved cranial nerves are the vagus and hypoglossal, followed by the cranial nerves affecting the ocular motor musculature, especially the abducens and trochlear, as well as the involvement of Horner's syndrome. Trigeminal sensory disturbance and trigeminal neuralgia have also been described.…”

Role of neural integrators in oculomotor systems: a systematic narrative literature review

“…; Nogués et al. ). This condition is compressive affecting the brainstem, including the hypoglossal nucleus with fluid‐filled cavities.…”

“…As NI have an immense network of connections with a vast array of structures, there may be presenting signs other than GEN. Additional neuroophthalmological findings have varied from impaired gaze holding, different types of nystagmus, ocular misalignment and vestibular imbalance (Table 1) (B€ uttner & Grundei 1995;Dieterich et al 2005;Rowe et al 2013;Shaikh & Ghasia 2013Kim et al 2014a). Cases reported have aetiologies such as infarctions and haemorrhages (Hommel & Bogousslavsky 1991;Halmagyi et al 1994;B€ uttner et al 2002;Helmchen et al 2002;Dieterich et al 2005;Rowe et al 2013;Kim et al 2014a), space-occupying lesions (Rett 2007;Karatas 2009;Lloyd et al 2009), syringobulbia (Weissman et al 1990;Nogu es et al 2010), Arnold-Chiari malformation (Weissman et al 1990;Wagner et al 2008;Ghasia et al 2014), demyelination (Averbuch-Heller et al 1995;Kim et al 2006Kim et al , 2014b, infection (Livorsi et al 2010), drug intoxications (Corbett et al 1989;Fischera et al 2009) and trauma (Sabates et al 1991;Van Stavern et al 2001;Kulkarni et al 2005;Odebode et al 2005). Additional investigations using imaging techniques such as CT or MRI scans have localized lesions within the brainstem and cerebellum, indicative of involvement of NI, including their abundant connecting structures which complete the gaze-holding system Helmchen et al 2002;Seo et al ...…”

“…Cases of patients with syringobulbia commonly report nystagmus with ocular and visual symptoms such as blurred vision and oscillopsia (Weissman et al 1990;Nogu es et al 2010). This condition is compressive affecting the brainstem, including the hypoglossal nucleus with fluid-filled cavities.…”