Cheiro-oral Syndrome Research Articles

P.071 Case report: cheiro-oral syndrome secondary to thalamic ischemic stroke

Background: Patient with small thalamic infarct may present with a variety of subtle and seemingly disconnected sensory deficits which can be easily missed. Methods: Here we report a case of an 80 years ‘old Chinese female presenting sudden onset persistent sensory symptoms over perioral area and right hand. The patient underwent further investigation with Brain MRI. The patient underwent further investigation with Brain MRI. A literature review was conducted and the patient’s clinical finding was compared with the literature. Results: The patient’s presentation was consistent with clinical manifestation of type 1 Cheiro-Oral syndrome. Brain MRI performed the day after admission revealed small non-hemorrhagic infarction involving the left thalamus. The diagnosis was type 1 cheiro-oral syndrome secondary to left thalamic ischemic stroke. Conclusions: This report highlights both clinical presentations of cheiro-oral syndrome and correlating clinical symptomatology with anatomic localization. It is important for physicians to recognize this rare condition with subtle presentations for complete work-up and definitive treatment.

Symptomatic hyperperfusion after combined revascularization surgery in patients with pediatric moyamoya disease: patient series

BACKGROUNDSymptomatic hyperperfusion after cerebral revascularization for pediatric moyamoya disease (MMD) is a rare phenomenon. The authors report a series of patients with this condition.OBSERVATIONSIn all three patients in this case series, the combined revascularization was on the left side, the patency of bypass grafts was confirmed after surgery, and focal hyperemia around the anastomotic site was observed on single photon emission computed tomography (SPECT). On the first to eighth days after surgery, all of the patients developed neurological manifestations, including motor aphasia, cheiro-oral syndrome, motor weakness of their right upper limbs, and severe headaches. These symptoms disappeared completely approximately 2 weeks after surgery, and all patients were discharged from the hospital. Quantitative SPECT was performed to determine the proportional change in cerebral blood flow (ΔRCBF) (to ipsilateral cerebellar ratio (denoted ΔRCBF) in the region of interest around the anastomoses, and the mean value was 1.34 (range, 1.29–1.41).LESSONSThis rare condition, which develops soon after surgery, requires an accurate diagnosis by SPECT. One indicator is that the ΔRCBF has risen to 1.3 or higher. Subsequently, strategic blood pressure treatment and fluid management could prevent the development of hemorrhagic stroke.

A case report: Numb Chin Syndrome due to thalamic infarction: a rare case

BackgroundNumb Chin Syndrome (NCS), which is also characterized as sensory neuropathy of the mental nerve, describes a mostly unilateral numbness of the chin and lower lip. Benign and malignant diseases are known to cause this circumscribed symptom, which can easily be overlooked or misdiagnosed. In this article we present the very rare case of a clinical NCS caused by thalamic lacunar infarction. As a pure sensory stroke it is a rare variant of the Cheiro-Oral Syndrome (COS).Case presentationA 63-year-old male patient received an emergency referral to our department after the patient had noticed a feeling of numbness of the left lower lip and chin on the previous day. The neurological examination revealed an approximately 2 × 3 cm area of hypoesthesia in the area of the chin and left lower lip and the cranial MRI an acute ischemia in the right thalamus.ConclusionsIn this case report we introduce a patient who clinically shows an NCS. Various diseases may be responsible for NCS, including malignancies or even central neurological disorders such as multiple sclerosis. A lacunar thalamic ischemia as a cause of NCS is very rare and to our knowledge described in the literature only in the contex of a COS in three cases. We wish to remind the reader, through this case, of the purely descriptive and syndromal character of the NCS and the importance for detecting underlying diseases. Furthermore we give a brief overview of the NCS and causative disorders.

Cerebral Hyperperfusion Syndrome After Revascularization Surgery in Moyamoya Disease: Region-Symptom Mapping and Estimating a Critical Threshold.

Cerebral hyperperfusion complicates the postoperative course of patients with moyamoya disease after direct revascularization surgery. There is no clear distinction between cerebral hyperperfusion syndrome and benign postoperative increase in regional cerebral blood flow (rCBF). The present study aimed to determine clinically relevant changes in rCBF, anatomical correlations, and factors associated with transient neurologic symptoms after revascularization surgery in moyamoya disease. Whole-brain voxel-based perfusion mapping was used to identify regions involved in cerebral hyperperfusion and quantify the changes in 105 hemispheric surgeries with the use of single-photon computed tomography acquired on postoperative day 7. The changes in rCBF were quantitatively analyzed, and associations with cerebral hyperperfusion syndrome were determined. Transient neurologic symptoms appeared with rCBF increase in 37.9% of adults. Speech impairments were associated with an increase in rCBF in the operculo-insula region. Cheiro-oral syndrome was associated with the posterior insula as well as the prefrontal region. A receiver operating curve analysis yielded transient neurologic symptoms with maximum accuracy at >15.5% increase from baseline. Age and preoperative rCBF were independently associated with transient neurologic symptoms (P < 0.001). Areas showing rCBF increase during the experience of transient neurologic symptoms were spatially compatible with the known functional anatomy of the brain. An increase of approximately 15% from baseline was found to be critical, which is a far lower threshold than what has been reported previously. Increasing age was significantly associated with the occurrence of symptomatic hyperperfusion. Furthermore, patients with preserved rCBF also showed symptomatic hyperperfusion.

Cheiro-oral syndrome: 1 case report

Cheiro-oral syndrome: 1 case report

Cheiro-Oral syndrome from lateral medullary infarction

Background: Although the variety of sensory impairments exists in lateral medullary infarction (LMI), cheiro-oral syndrome (COS) has rarely been reported.

TNF-alpha inhibitor therapy associated demyelination

Objectives Tumour necrosis factor alpha (TNFα) inhibitor therapy has been documented to be associated with demyelinating lesions, including transverse myelitis and optic neuritis sometimes characteristic of multiple sclerosis (MS). It has not previously been reported to mimic lesions typical for neuromyelitis optica spectrum disorder (NMOSD). We present three cases of demyelination associated with TNFα inhibitor therapy, one case developed cheiro-oral syndrome suggestive of NMOSD and two developed transverse myelitis. Methods Patients who presented with first episodes of demyelination whilst receiving TNFα inhibitor therapy were identified form clinical records. Cases A 65-year-old woman of Vietnamese ancestry, immediately after receiving her first dose of certolizumab, developed paraethesiae of her lips, tongues, hands and feet associated with nausea and mild ataxia. Examination identified reduced sensation of the perioral region and tongue. MRI of the brain revealed signal abnormality centred in the mid brain at the level of interpeduncular notch. Repeat MRI two months after cessation of certolizumab showed resolution of the lesion, with corresponding symptomatic improvement. Aquaporin-4 antibodies were not detected. A 63 years old female presented with lower limb sensory disturbance after first dose of infliximab for rheumatoid arthritis. Examination identified bilateral leg weakness, urinary retention and a sensory level to the upper abdomen. MRI revealed an isolated T8/9 lesion. A 43-year-old-female presented 2 years after commencing adalimumab with complaints of subtle left leg weakness. Examination revealed hyperreflexia and an extensor plantar response. MRI showed lesions at C2 and T9. In both cases CSF was normal and negative for oligoclonal bands. Follow-up ranges from 1 to 14 years and since discontinuation of TNF-alpha therapy no further episodes of demyelination have occurred. Conclusions TNFα therapy is associated with demyelination, commonly with normal CSF analysis. Discontinuation of therapy is recommended in patients presenting with TNFα therapy associated demyelination.

Crossed cheiro-oral syndrome: A warning sign for medullary involvement and neurological deterioration

Crossed cheiro-oral syndrome (CCOS) is characterized by crossed sensory disturbance confined to the unilateral perioral area and contralateral hand/finger(s). Although a few classical crossed sensory syndromes accurately predict brainstem or spinal involvement, the clinical significance of CCOS remains unclear. In this study, we analyzed the etiology, localization and outcome of CCOS patients. The results showed that ischemic stroke is the exclusive cause of CCOS. The location of responsible stroke is pertinent to the middle or upper level of the lateral medulla oblongata medial to the lateral sulcus. The vascular supply is from the vertebral artery or the posterior inferior cerebellar artery. Half of the CCOS patients progressed to Wallenberg’s syndrome and complicated with disabled daily living. However, no patient died during the follow-up period. A larger size and dorsal extension of the infarction correlated with neurological deterioration. Therefore, CCOS is an independent clinical sign of medullary involvement. It strongly predicts involvement at the lateral medulla oblongata, especially the ischemic stroke, and neurological deterioration. A rapid evaluation of the infarction location and vascular status is suggested in cases of CCOS.

Cheiro-Oral Syndrome Secondary to Thalamic Infarction

Small thalamic infarcts can present with a variety of sensory deficits that can be difficult to diagnose clinically because of their seemingly disconnected manifestations. Here we report a case of a 55-year-old man presenting with the clinical findings of Cheiro-Oral syndrome, a pure sensory thalamic lacunar syndrome that clinically presents with contralateral sensory deficits of the fingers and hemimouth. This report highlights both common and unusual clinical presentations of isolated thalamic infarctions, correlating clinical symptomatology with anatomic localization. In patients with such findings on examination, thalamic infarction should be considered, with a thorough workup initiated to both confirm the diagnosis and optimally reduce future stroke risk.

Cheiro-oral syndromeを呈した皮質性梗塞の1例

大脳皮質の体性感覚野に限局した小梗塞によりcheiro-oral syndrome （COS）を呈した1例を報告する．症例は46歳女性．右中心前回の小梗塞による左口角周囲の顔面麻痺の既往あり．今回は左鼻翼部外縁から口角，左手指のしびれで発症した．頭部MRIで右中心溝底部から中心後回皮質に小梗塞を認め，SEP右正中神経刺激でN20は正常であることから，Brodmann 3a （中心溝底部）を主病巣とする皮質性COSと診断した．抗血小板剤により症状は軽快した．前回および今回の梗塞巣の分布から，中大脳動脈皮質枝の中心溝動脈に限局したアテローマが存在し，血栓症またはA to A塞栓症を生じたものと推察した．最近の多数例の検討では，COSは延髄から体性感覚野に至る感覚伝道路を障害する様々な病因により生じ，症状増悪例も報告されており，症例ごとに慎重に原因検索を行い，病態に応じた治療を行う必要がある．

Spinal cheiro-oral syndrome: a common neurological entity in an unusual site

Background and purposeCheiro-oral syndrome (COS) is an established neurological entity characterized by a sensory impairment confined to the mouth angle and ipsilateral finger(s)/ hand. The current understanding of localization is a concomitant involvement of the spinothalamic and trigeminothalamic tract between the cortex and pons. The cervical spinal cord has not been mentioned in this situation yet, and this unusual location may heretofore increase the risk of misdiagnosis. Material and methodsSix patients who presented with unilateral COS due to cervical cord disorder are reported. ResultsAll patients were women and their age ranged between 42 and 70 years. Their neurological deficits included unilateral paraesthesiae restricted to cheiro-oral distribution, positive radicular sign, and mild change of tendon reflex. Cervical spinal stenosis at middle/lower cervical spine with variable magnitude of cord compression and intrinsic cord damage was found. A diagnostic dilemma obviously arises from the lack of tangible neurological signs or typical pattern of myelopathy, in addition to the previous concept of cerebral involvement. A benign course ensued in all reported patients. ConclusionsCheiro-oral syndrome can be an early neurological sign for cervical cord disorder; it further suggests that it is a strong neurological but weak localizing sign. A reciprocal influence of multiple factors is considered to generate COS at the cervical cord. Therefore, an absence of brain pathology should lead to a thorough examination of the cervical cord in case of COS.

Cheiro-oral syndrome associated with lateral medullary infarction

A 62-year-old man was admitted to our department because of the sudden onset of dysaesthesia in the right perioral region of the face and right hand. Neurological examination demonstrated a loss of pain and temperature sensation in both the right perioral region and in the fingers of the right hand. These findings suggested damage to parts of the ipsilateral spinothalamic and trigeminothalamic ascending tracts, producing restricted sensory impairment of the perioral region and ipsilateral hand. This is the first reported case of a medullary lesion causing cheiro-oral syndrome.

Incidence and Pathogenesis of Transient Cheiro-Oral Syndrome After Surgical Revascularization for Moyamoya Disease

There are no reports that denote transient cheiro-oral syndrome (COS) after surgical revascularization for moyamoya disease. To clarify the incidence and pathogenesis of transient COS after surgical revascularization for moyamoya disease. This study included 21 patients who underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and indirect bypass because of Moyamoya disease. Their medical records were evaluated to identify clinical features of postoperative transient COS. The findings on MRI, magnetic resonance angiography, and single-photon emission computed tomography were also analyzed. Transient COS developed in 8 (22.9%) of 35 operated hemispheres, or in 6 (28.6%) of 21 patients between 3 and 20 days after surgery. Most of the COS were associated with mild weakness of the ipsilateral face and hand. Simultaneous radiological studies detected no findings of cerebral infarct or postoperative hyperperfusion. STA-MCA anastomosis was patent in all patients. However, their disease stage more frequently progressed owing to considerable blood flow via STA-MCA anastomosis, and basal moyamoya vessels disappeared or diminished in patients with transient COS rather than in those without. Transient COS after surgical revascularization for moyamoya disease is not rare. Bypass flow through STA-MCA anastomosis may stimulate a rapid progression of disease stage and diminish basal moyamoya vessels, causing transient COS within 3 weeks after surgery.

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

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.

Crossed cheiro-oral syndrome

Objectives Cheiro-oral syndrome is characterized by sensory impairment confined to perioral area and ipsilateral fingers/hand. It results from an involvement of the ascending sensory tracts above the pons. However, a crossed pattern of perioral and acral paresthesia was rarely reported before. Patients and methods This study reports the neuroanatomic relationship, course and clinical significance of perioral and contralateral acral paresthesia in four patients. We term it the crossed cheiro-oral syndrome. Results All patients had lateral or dorsolateral medullary infarctions that were ipsilateral to their perioral paresthesia. The contributory origin is considered a diagonal lesion involving the par oralis fibers within the descending trigeminal sensory tract and acral portion of the lateral spinothalamic tract at the lateral portion of medulla oblongata. Despite of a restricted sensory disturbance at initial, progressive neurological disability terminated to Wallenberg's syndrome ensued in three patients and disabling deficits persisted in two of them. Conclusion The crossed cheiro-oral syndrome seems a mild form of Wallenberg's syndrome. Therefore, it predicts medullary involvement and is also a warning sign for progression.

Commentary

Cerebral hyperperfusion complicates the postoperative course of patients with moyamoya disease after direct revascularization surgery. There is no clear distinction between cerebral hyperperfusion syndrome and benign postoperative increase in regional cerebral blood flow (rCBF).The present study aimed to determine clinically relevant changes in rCBF, anatomical correlations, and factors associated with transient neurologic symptoms after revascularization surgery in moyamoya disease.Whole-brain voxel-based perfusion mapping was used to identify regions involved in cerebral hyperperfusion and quantify the changes in 105 hemispheric surgeries with the use of single-photon computed tomography acquired on postoperative day 7. The changes in rCBF were quantitatively analyzed, and associations with cerebral hyperperfusion syndrome were determined.Transient neurologic symptoms appeared with rCBF increase in 37.9% of adults. Speech impairments were associated with an increase in rCBF in the operculo-insula region. Cheiro-oral syndrome was associated with the posterior insula as well as the prefrontal region. A receiver operating curve analysis yielded transient neurologic symptoms with maximum accuracy at >15.5% increase from baseline. Age and preoperative rCBF were independently associated with transient neurologic symptoms (P < 0.001).Areas showing rCBF increase during the experience of transient neurologic symptoms were spatially compatible with the known functional anatomy of the brain. An increase of approximately 15% from baseline was found to be critical, which is a far lower threshold than what has been reported previously. Increasing age was significantly associated with the occurrence of symptomatic hyperperfusion. Furthermore, patients with preserved rCBF also showed symptomatic hyperperfusion.

視床性手口感覚症候群の臨床的検討

We report 6 patients with Cheiro-oral syndrome (COS), with special reference to clinical features and responsible lesions. The time intervals from the onset of symptoms to arrival in our department were less than 24 hours in 3 patients, 2 days in 2, and 5 days in 1. All patients had subjective sensory disturbance involving the unilateral hand and ipsilateral perioral regions, and 4 patients presented with objective sensory disturbance. The body parts of tingling sensation tended to be larger than those of superficial sensory disturbance. Three patients developed motor disturbance including hemiparesis with or without ataxia, clumsiness of fine finger movements, and dysarthria. Magnetic resonance imaging revealed fresh infarctions around the thalamus, including lacunar infarctions in 5 patients and branch atheromatous disease in 1 patient. The lesion sites responsible for COS were ventral posterolateral (VPL) and ventral posteromedial (VPM) nuclei in the thalamus in 4 patients, thalamic pulvinar nucleus and medial geniculate body in 1, thalamic ventroposterior region-internal capsule-corona radiata in 1. Three patients had asymptomatic brain infarctions. Risk factors were hyperlipidemia, hypertension, diabetes mellitus, smoking, arteriosclerosis of the carotid artery, and polycythemia. In the convalescent stage, 5 patients suffered from residual sensory-motor disturbance, whereas 1 patient recovered from COS. COS has been attributed mainly to small infarctions in the thalamic ventroposterior nuclei. However, it is suggested that damage to ascending sensory fibers projecting to the thalamic VPL and VPM nuclei can cause COS. Because initial symptoms of COS are apt to be overlooked, early diagnosis and treatment are necessary to avoid residual sensory-motor disturbance.

Cortical cheiro-oral syndrome: A revisit of clinical significance and pathogenesis

Objectives A restricted neurological deficit is not uncommon in patients with cortical lesions. However, in cheiro-oral syndrome (COS) associated with cortical involvement, the topographic disparity of the cheiral and oral representation area is hardly explained by the restricted sensorium at the homolateral mouth angle/lip and finger/hand, with sparing of the facial structures. Patients and methods In order to elucidate the factors of clinical significance and pathogenesis in cortical COS, 11 patients with COS associated with contralateral cortical lesions were analyzed, and the cases of similar patients described in the literature were reviewed. Results In our patients, the etiologies of COS were infarct, subdural hematoma, meningioma, and vascular malformation. The lesions were sizable enough to involve areas beyond the postcentral gyrus of the parietal lobe. A cortical sensory sign was detected in only six patients. Their paroxysmal sensorium without exacerbation on stimulus was in contrast to the clinical features of COS associated with thalamic lesions. Five patients suffered worsening of their neurological deficits without a parallel expansion of their infarct or hemorrhage. Removal of the hematoma or tumor in four patients rapidly abolished their sensorium. Conclusion Cortical COS is a warning sign of a potentially life-threatening etiology. Since there is a high frequency of exacerbation, COS should be carefully investigated, as a paucity of associated clinical signs was found in our patients. Paroxysmal sensorium signifies possible cortical involvement. Rapid decompression within the “golden period” is encouraged. It seems that the interaction between previously existing vascular compromise, sensory plasticity, and neuronal vulnerability predisposes patients to this peculiar sensory disorder. Epileptogenesis is unlikely. Thus, a reconsideration of COS in clinical practice is warranted.

Clinical study of 99 patients with pure sensory stroke

We report a clinical description of pure sensory stroke based on data collected from a prospective acute stroke registry. From 2500 acute stroke patients included in a hospital-based prospective stroke registry over a 12-year period, 99 were identified as having pure sensory stroke. Pure sensory stroke accounted for 4.7% of all acute stroke patients, 5.4% of acute ischemic stroke, and 17.4% of lacunar syndromes. Complete hemisensory syndrome was present in 80 patients and incomplete hemisensory syndrome in 19 (cheiro-oral syndrome 12, cheiro-oral-pedal 6, isolated oral syndrome 1). The lacunar hypothesis was fulfilled in 88% of patients. Atherothrombotic infarction occurred in 8 patients, intracerebral hemorrhage in 3, and stroke of undetermined cause in 1. Hemorrhagic pure sensory stroke was diagnosed in 1% of all cases of hemorrhagic stroke (n = 270) in the database. Outcome was good (in-hospital mortality 0%, symptom-free at discharge 41.5%). After multivariate analysis, absence of disability at discharge, hypertension, diabetes, hyperlipidemia, and thalamic (56.5%) and corona radiata (4%) locations were clinical and topographic variables significantly associated with pure sensory stroke. Pure sensory stroke is an infrequent cerebrovascular syndrome, in which the lacunar hypothesis is supported. Most patients had thalamic lacunar infarction. Incomplete hemisensory syndromes were also caused by a lacunar infarct in 84% of patients. Hemorrhagic pure sensory stroke accounted only for 3% of the cases. The prognosis is good with striking similarity to other lacunar strokes. There are important differences between pure sensory stroke and nonlacunar strokes.

Síndrome sensitivo puro queiro-oro-podal: estudio de 17 pacientes

To characterize the clinical features, etiology and prognosis in cheiro-oral-pedal syndrome. Descriptive study of 17 patients with cheiro-oral-pedal syndrome included in the Sagrat Cor Hospital of Barcelona Stroke Registry over a 11 year period. Cheiro-oral-pedal syndrome was present in 6 patients (35%), cheiro-oral syndrome in 10 (59%) and isolated oral syndrome in 1 (6%). Cheiro-oral-pedal syndrome was caused by a lacunar infarct in 88% of patients and by atherotrombotic infarcts in 12%. Cheiro-oral-pedal syndrome accounted for 0.7% of all acute strokes (n = 2.244), 1% of all cerebral infarcts (n = 1.649), 3.5% of all lacunar infarcts (n = 422) and 18.5% of all pure sensory lacunar infarcts (n = 80). Thalamus (n = 13), internal capsule (n = 2), striatocapsular involvement (n = 1) and fronto-parietal involvement (n = 1) were the cerebral topographies. Absence of in hospital mortality and absence or mild neurological deficit at discharge from the hospital were present in all the patients. Lacune hypothesis is present in cheiro-oral-pedal syndrome. Cheiro-oral-pedal syndrome was caused by a lacunar infarct in 88% of patients. The thalamic topography is the most frequent and the prognosis in cheiro-oral-pedal syndrome is good.