Chiari I malformation associated with atlanto-occipital assimilation presenting as orthopnea and cough syncope

2017 ACC/AHA/HRS guideline for the evaluation and management of patients with syncope

“…Rarely associated with complete heart block and syncope. Syringomyelia [590][591][592][593][594][595][596][597] Chiari malformation 598 Arnold Chiari malformations are the most common form of syringomyelia.…”

Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management

“…Other common presenting symptoms include nausea, dysphagia, apnoea, clonus, cerebellar symptoms, drop attacks, muscle atrophy and dysphonia ( Table 5). Case reports exist of more unusual presentations such as orthopnoea 18 , vertigo 19 and diplopia 20 . Additionally, a number of patients are found incidentally on MRI scans.…”

“…21 The literature search found 21 papers with details on the presenting symptoms of one or more cases of CM-1. [4][5][6][8][9][10]15,16,[19][20][21][22][23][24][25][26][27][28][29][30] Of these, 13 discussed fewer than 10 patients, 6,8,10,15,16,[18][19][20]22,[25][26][27][28] with the majority of these being case reports. Four looked at between 10 and 40 patients 3,9,24,29 and three looked at over 40 patients.…”

“…While sleep apnoea is seen in a small proportion of patients, Mangubat et al recorded a case of orthopnoea due to CM-1. 18 Klein et al reported a case of vertigo and vestibular difficulties 19 and Furuya et al reported six cases of vertigo. 29 Patients can also present with visual problems.…”

Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery

“…Some authors have reported that syncope presents as a symptom in only 3% of CM-I patients12). Classically, patients with CM-I present with pain, weakness, sensory loss, dizziness, gait disturbances, and cranial nerve palsies.…”