Cheiro-Oral Syndrome: A Clinical Analysis and Review of Literature

Abstract

PurposeAfter a century, cheiro-oral syndrome (COS) was harangued and emphasized for its localizing value and benign course in recent two decades. However, an expanding body of case series challenged when COS may arise from an involvement of ascending sensory pathways between cortex and pons and terminate into poor outcome occasionally.Materials and MethodsTo analyze the location, underlying etiologies and prognosis in 76 patients presented with COS collected between 1989 and 2007.ResultsFour types of COS were categorized, namely unilateral (71.1%), typically bilateral (14.5%), atypically bilateral (7.9%) and crossed COS (6.5%). The most common site of COS occurrence was at pons (27.6%), following by thalamus (21.1%) and cortex (15.8%). Stroke with small infarctions or hemorrhage was the leading cause. Paroxysmal paresthesia was predicted for cortical involvement and bilateral paresthesia for pontine involvement, whereas crossed paresthesia for medullary involvement. However, the majority of lesions cannot be localized by clinical symptoms alone, and were demonstrated only by neuroimaging. Deterioration was ensued in 12% of patients, whose lesions were large cortical infarction, medullary infarction, and bilateral subdural hemorrhage.ConclusionCOS arises from varied sites between medulla and cortex, and is usually caused by small stroke lesion. Neurological deterioration occurs in 12% of patients and relates to large vessel occlusion, medullary involvement or cortical stroke. Since the location and deterioration of COS cannot be predicted by clinical symptoms alone, COS should be considered an emergent condition for aggressive investigation until fatal cause is substantially excluded.

Clinical analysis of cheiro-pedal syndrome

“…However, isolated CPS is uncommon, and we have encountered only nine patients within the enrollment period of our study, in comparison with more than 70 patients with COS. 3 This may relate to the strict diagnostic criteria of CPS, in that the cheiral and pedal paresthesia must occur abruptly, concomitantly, and locally without other neurological deficits. Nevertheless, this strict criteria helps to define the neuroanatomy and etiopathogenesis of this peculiar sensory disorder from other forms of RASS and distal paresthesia.…”

“…The only exception is in the thalamus at where the Since CPS is similar to COS except for absence of the perioral paresthesia, they may share some common pathogenesis. 3 In COS, a site-specific pathogenesis has been proposed. A close proximity of cheiral, pedal and oral sensory fibers in the ventral trigeminothalamic tract is suggested for COS at the pons, whereas a cooperative interaction of neuronal vulnerability, 19 neuronal spasticity, 20,21 and preconditioned damage is suggested at the somatosensory cortex.…”

“…3,8 In our database, we defined CPS strictly as an acute onset of a subjective and/or objective sensory disturbance confined to the ipsilateral hand/finger(s) and foot/toe(s) simultaneously, without a detectable abnormality of mental, memory, motor or cerebellar function. 3 The following forms of cheiro-pedal paresthesia were excluded: (i) a residual sensory deficit recovered from a more extensive sensory impairment; (ii) that associated with an ictus in migraine; (iii) a symptom of hyperventilation syndrome; (iv) an occurrence shortly after traumatic brain injury, marine intoxication, or dermatological diseases such as hand-foot erythrodysesthesia, or glovesand-socks syndrome in viral infection.…”

“…1 The family of restricted acral sensory syndromes (RASS) consists of a constellation of incomplete sensory disorders confined to the distal parts of the limbs and/or face. 1,2 This may occur at a single site or multiple, distantly separated sites, 2 including cheiro-oral syndrome (COS), 3 cheiro-oral-pedal syndrome, and their variants. Cheiro-pedal syndrome (CPS) also belongs to the RASS, and its sensory deficit is confined to the hand/finger(s) and ipsilateral foot/toe(s).…”

Underlying Causes of Paresthesia

“…In another case report, a thalamic lacunar infarct led to sudden isolated left-sided paresthesia involving the face, upper and lower limbs (28). Cheiro-oral syndrome is characterized by paresthesia and sensory impairment confined to the perioral region and ipsilateral fingers or hand, and arises from small stroke-related lesions to various sites between the medulla and cortex (29).…”