Atypical Cheiro-oral Syndrome Presented With Bilateral Perioral Sensory Symptoms in Unilateral Thalamic Infarction

Cheiro-oral syndrome is a unilateral sensory impairment that is restricted to the homolateral hand and perioral area. An unusual clinical presentation with bilateral perioral involvement has also been described. Most of the responsible lesions involved the paramedian pontine tegmentum; thalamic involvement is extremely rare. We report herein a patient who presented with purely sensory symptoms involving the bilateral perioral regions and the distal thumb and index finger of the left hand following a small infarction in the right lateral thalamus.

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

PURPOSE: After 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 METHODS: To analyze the location, underlying etiologies and prognosis in 76 patients presented with COS collected between 1989 and 2007. RESULTS: Four 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. CONCLUSION: COS 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.

Clincal, MRI and MRA Findings of Cheiro-Oral-Pedal syndrome

BACKGROUND: Cheiro-oral-pedal syndrome is characterized by a unilateral or bilateral sensory disturbance in mouth corner and hand and/or foot, and associated with central nervous system disease. Causative diseases are cerebral infarction, intracranial hemorrhage, brain tumor, migraine, etc. When related with cerebral infarction, it is known to have lacunar type pathology and favorable prognosis. But other pathogenic mechanism and poor prognosis is suggested. METHODS: We retrospectively studied 12 patients presented with cheiro-oral-pedal syndrome, who visited the department of neurology in Chosun University Hospital from September 2000 to August 2002. Cheiro-oral-pedal syndrome was diagnosed according to the clinical manifestations and brain MRI findings. We assessed neurological findings, brain MRI and MRA findings, duplex sonographic findings, risk factors of stroke and outcome of the patients. Followup period was 3-20 (mean 8) months. RESULTS: There were 12 patients (10 men, 2 women) with the age ranged from 45 to 80 (mean 57.8) years. Sensory loss on cheiro-oral area was present in 8 (66.7%) and on cheiro-oral-pedal area in 4 (33.3%). Responsible lesions were found in thalamus, capsulo-striatum, corona radiata, pons, and fronto-parietal cortex. Three patients had a major cerebral vascular abnormality on MRA. One had MCA stenosis, the other PCA stenosis and another distal ICA stenosis. Sensory symptoms had remained in all patient except one. In-hospital mortality and other neurological deficit at discharge were absent in all. CONCLUSIONS: Cheiro-oral syndrome has mixed pathogenic mechanism of small vessel disease and large vessel atherosclerosis and involves various sites. Paresthesia itself has bad prognosis but overall clinical course is benign.

Cheiro-oral Syndrome with INO following Brainstem Infarction

Brainstem lesions may produce a cheiro-oral syndrome infrequently. The cheiro-oral syndrome in pontine lesion is due to involvement of ventral trigeminothalamic tract and the medial part of the medial lemniscus. The cheiro-oral syndrome rarely associates with internuclear ophthalmoplegia. A 58-year-old hypertensive man complained of dizziness, diplopia, and dysesthesia in the right hand and ipsilateral perioral region. On neurologic examination, there was left internuclear ophthalmoplegia. Others were unremarkable. Brain MRI demonstrated a small round lesion in the paramedian tegmentum of the left upper pons and lower midbrain, which involved ventral trigeminothalamic tract, medial part of medial lemniscus and medial longitudinal fasciculus. We report a case of brainstem infarction with a rare combination of cheiro-oral syndrome and internuclear ophthalmoplegia.