Unilateral Facial Nerve Research Articles

Diagnostic utility of Freyss motor examination, House–Brackmann grading, topognostic tests, and electrophysiological assessments for unilateral peripheral facial nerve disorder

Unilateral peripheral facial nerve disorder is most often idiopathic, and the determination of the cause may be necessary to guide appropriate treatment. There is no standard diagnostic test for determining the cause or location of the lesion; therefore, clinicians may rely on a variety of diagnostic methods such as the Freyss system of motor examination, House–Brackmann grading, topognostic tests (Schirmer, stapedius reflex, gustatometry), and electrophysiological investigations (nerve conductivity, blink reflex, needle EMG). However, few studies have directly compared the suitability of these different diagnostic methods according to disease characteristics (severity, onset, etc.). This descriptive cross-sectional study compared results using these diagnostic modalities among 44 consecutive patients. There was a significant concordance (Kappa R = 0.5, p < 0.05) between the Freyss motor system examination results and House–Brackmann scoring for 32 patients with chronic onset and moderate-to-severe damage. Alternatively, there was poor agreement (Kappa R = 0.011, p = 0.935) between topognostic and electrophysiological investigation for determining lesion location. In 13 patients with chronic onset and moderate-to-severe damage, lesion location could not be determined based on electrophysiology, while topognostic examination was able to determine the location for both acute- and chronic onset cases. For lesions that cannot be assessed by electrophysiology, Freyss and House–Brackmann motor tests can be used for diagnoses and prognosis.

Möbius syndrome: clinico-radiologic correlation.

Möbius syndrome is characterized by abducens and facial nerve palsy. However, the presence/absence of corresponding cranial nerves on MRI was not fully evaluated. The purpose of this study was to investigate the mechanism of Möbius syndrome by associating the presence of abducens and facial nerves on MR imagings with clinical features. We retrospectively reviewed the medical records and MR imagings of nine patients with Möbius syndrome between January 2004 and October 2015. The presence/absence of abducens and facial nerves on MR imaging, as well as corresponding neuro-ophthalmologic clinical features, was investigated. Facial palsy was bilateral in six and unilateral in three patients. Abduction was limited bilaterally in five and unilaterally in four patients. The degrees of facial palsy and abduction limitation were variable, and asymmetric even in the bilateral cases. MR imaging documented bilateral absence of the abducens and facial nerves in six, absence of unilateral facial nerve and bilateral abducens nerves in one, and absence of facial and abducens nerves unilaterally on the same side in another. Both abducens and facial nerves were visualized bilaterally only in the one remaining patient. The absence of abducens and facial nerves on MR imaging was mostly correlated with the findings of facial palsy and abduction limitation in patients with Möbius syndrome. MR imaging aids in diagnosis of Möbius syndrome by documenting the absence or presence of abducens and facial nerves.

Facial Asymmetry Index: Validation and Applications in Various Smile Restoration Techniques.

Consistent and objective evaluation of the paralyzed face is imperative for documenting preoperative findings and assessing postoperative outcomes of reanimation techniques. Static and dynamic facial asymmetry are the key features of the paralyzed face. To date, there is no consensus among surgeons on how best to document facial asymmetry. The authors propose a Facial Asymmetry Index (FAI) as an objective measure of facial asymmetry and validate its use and reliability across numerous reanimation techniques. Frontal photographs of patients with unilateral facial nerve paralysis were analyzed. The length from the medial canthus to the ipsilateral oral commissure was compared between affected and nonaffected sides. The FAI is the difference between the two values, with a higher value reflecting poorer facial symmetry. Validation and reliability testing was then performed. There was a consistent decrease in the FAI with procedural intervention (FAI pre = 10.1, FAI post = 3.17; p < 0.0001). Furthermore, there was excellent inter- and intrarater reliability among independent judges. The FAI is a powerful and accessible tool to quantify operative outcomes for many lower facial nerve reanimation techniques.

Facial nerve palsy and laryngospasm as a complication of local anaesthesia during adenotonsillectomy

Tonsil surgeries are the most frequently performed surgical procedures in ENT departments. We would like to present the case of a 5-year-old patient who suffered from unilateral peripheral facial nerve palsy and laryngeal spasm following adenotonsillectomy. Paresis was observed immediately after the transfer of the patient to the postoperative room. The activity of facial muscles was restored within 2 hours from the beginning of the surgery. We assume that this was the direct effect of an anaesthetic on the extracranial processes of the facial nerve.

Nonsurgical Treatment for Posttraumatic Complete Facial Nerve Paralysis

ImportanceCurrent recommendations envisage early surgical exploration for complete facial nerve paralysis associated with temporal bone fracture and unfavorable electrophysiologic features (response to electroneuronography, <5%). However, the evidence base for such a practice is weak, with the potential for spontaneous improvement being unknown, and the expected results from alternative nonsurgical treatment also undefined.ObjectiveTo document the results of nonsurgical treatment for posttraumatic complete facial paralysis with undisplaced temporal bone fracture and unfavorable electrophysiologic features.Design, Setting, and ParticipantsProspective cohort study recruiting from April 2010 to April 2013 at a tertiary care university hospital. Follow-up continued until 9 months or until complete recovery if earlier. Study group included 28 patients with head injury–associated complete unilateral facial nerve paralysis with unfavorable results of electroneuronography (<5% response) with or without undisplaced temporal bone fracture. Undisplaced temporal bone fractures were documented in 26 patients (24 longitudinal fractures and 2 transverse fractures).InterventionsPatients received prednisolone, 1 mg/kg, for 3 weeks combined with clinical monitoring every 2 weeks and electromyography monitoring every 4 weeks. As per study protocol, surgical exploration was limited to patients demonstrating motor end plate degeneration on results of electromyography, or having no improvement until 18 weeks.Main Outcomes and MeasuresFacial nerve function was evaluated by the House-Brackmann grading system; Forehead, Eye, Mouth, and Associated defect grading system; and the modified Adour system. Observations were completed at 40 weeks.ResultsAmong the 28 patients in the study (3 women and 25 men; mean [SD] age, 32.2 [8.7] years), facial nerve recovery with conservative treatment alone was noted in all patients. No recovery was seen in any patient at the initial 4-week review. The first signs of clinical recovery were noted in 4 patients by 8 weeks, in 27 patients by 12 weeks, and in all patients by 20 weeks. No patient required surgical exploration. At 40 weeks, 27 patients recovered to House-Brackmann grade I/II and 1 patient to grade III. All 24 patients with longitudinal fractures had grade I/II recovery.Conclusions and RelevanceFor undisplaced temporal bone fractures, nonsurgical treatment leads to near-universal recovery to House-Brackmann grade I/II and is superior to reported surgical results. Recovery is delayed and usually first manifests at 8 to 12 weeks after the fracture. In the current era of high-resolution computed tomography, surgical exploration should not be first-line treatment for undisplaced longitudingal temporal bone fractures associated with complete facial nerve paralysis and unfavorable electrophysiologic features.

Facial nerve stimulation in normal pigs and healthy human volunteers: transitional development of a medical device for the emergency treatment of ischemic stroke

BackgroundMagnetic stimulation of the facial nerve has been tested in preclinical studies as a new, non-invasive emergency treatment of ischemic stroke that acts by increasing cerebral blood flow (CBF). The objective of the studies reported herein was to identify minimal stimulation parameters that increase CBF in large animals and then test those stimulation parameters in healthy volunteers for safety, tolerability, and effectiveness at increasing CBF. This translational research is necessary preparation for clinical studies in ischemic stroke patients.MethodsInitial experiments in anesthetized Yorkshire pigs were undertaken in order to identify the lowest stimulus power and duration that increase CBF. A full 3 × 3 factorial design was used to evaluate magnetic stimulation of the facial nerve at various stimulation powers (1.3, 1.6, and 1.9 Tesla field strength at coil surface) and for various durations (2, 3.5, and 5 min). CBF was measured with contrast MRI perfusion imaging and the internal carotid arteries were assessed with MR angiography. Magnetic facial nerve stimulation with parameters identified in the pig study was then applied to 35 healthy volunteers. Safety was assessed with adverse event reports and by medical examination. Tolerability was defined as each volunteer’s ability to withstand at least 2 min of stimulation. Volunteers could determine the maximum power of stimulation they received during a ramp-up period.ResultsIn pigs, unilateral facial nerve stimulation increased CBF by as much as 77% over pre-stimulation baseline when administered across a range of 1.3–1.9 Tesla power and for 2- to 5-min duration. No clear dose–response relationship could be observed across this range, but lower powers and durations than these were markedly less effective. The effect of a single stimulation lasted 90 min. A second stimulation delivered 100 min after the first stimulation sustained the increased CBF without evidence of tachyphylaxis. In human, bilateral facial nerve stimulation caused only non-serious adverse events that were limited to the 2-min stimulation period. Tolerability was greatly improved by gentle encouragement from the study staff, which enabled most volunteers to tolerate 1.6–1.8 Tesla of stimulation power. CBF measures taken approximately 10 min after stimulation demonstrated on average a 32 ± 6% increase in CBF, with ≥ 25% increases in CBF occurring in 10 of the 31 volunteers who had adequate CBF measurements.ConclusionsThe minimal effective stimulation parameters defined by increased CBF, as identified in the pig study, translated into safe, tolerable, and effective stimulation of healthy volunteers. These results support the future development and evaluation of non-invasive facial nerve stimulation for the emergency treatment of ischemic stroke.Trial Registration retrospectively registered with clinicaltrials.gov NRV_P1_01_15 on June 6, 2017

The role of Hedgehog-responsive fibroblasts in facial nerve regeneration

BackgroundFacial nerve paralysis is a significant cause of morbidity, affecting facial appearance, emotional expression, speech, oral competence, and vision. A more complete understanding of the complex cellular events required for successful nerve regeneration may reveal new therapeutic targets. The role of fibroblasts in regeneration, and the process by which the nerve reforms its three-dimensional structure after a transection injury, are not fully understood. The Hedgehog signaling pathway has been shown to mediate nerve sheath formation during development. We therefore sought to characterize the role of Hedgehog-responsive cells following transection of the facial nerve. MethodsTwo transgenic mouse lines with reporters for the downstream effector of Hedgehog signaling, Gli1, were used. The animals underwent a unilateral facial nerve transection injury, and the contralateral side served as a control. Facial nerves were analyzed via immunohistochemistry and immunofluorescence at predetermined time points as the facial nerve regenerated after the transection injury. ResultsThere was a statistically significant increase in Gli1+ cells both at the site of injury and within the distal nerve segment over time. Gli1+ cells are fibroblasts within the nerve and appear to contribute to the reformation of the nerve sheath after injury. ConclusionThese findings describe a key signaling pathway by which fibroblasts participate in motor nerve regeneration. Fibroblasts that reside within the nerve respond to injury and may represent a novel therapeutic target in the context of facial nerve regeneration after transection injury.

Individual Clinically Diagnosed with CHARGE Syndrome but with a Mutation in KMT2D, a Gene Associated with Kabuki Syndrome: A Case Report.

We report a Japanese female patient presenting with classic features of CHARGE syndrome, including choanal atresia, growth and development retardation, ear malformations, genital anomalies, multiple endocrine deficiency, and unilateral facial nerve palsy. She was clinically diagnosed with typical CHARGE syndrome, but genetic analysis using the TruSight One Sequence Panel revealed a germline heterozygous mutation in KMT2D with no pathogenic CHD7 alterations associated with CHARGE syndrome. Kabuki syndrome is a rare multisystem disorder characterized by five cardinal manifestations including typical facial features, skeletal anomalies, dermatoglyphic abnormalities, mild to moderate intellectual disability, and postnatal growth deficiency. Germline mutations in KMT2D underlie the molecular pathogenesis of 52–76% of patients with Kabuki syndrome. This is an instructive case that clearly represents a phenotypic overlap between Kabuki syndrome and CHARGE syndrome. It suggests the importance of considering the possibility of a diagnosis of Kabuki syndrome even if patients present with typical symptoms and meet diagnostic criteria of CHARGE syndrome. The case also emphasizes the impact of non-biased exhaustive genetic analysis by next-generation sequencing in the genetic diagnosis of rare congenital disorders with atypical manifestations.

Validation of CADS Grading Scale: An Ophthalmic Specific Grading Instrument for Facial Nerve Palsy.

To evaluate the inter- and intraobserver reliability of the CADS score, a previously described facial nerve grading instrument for ophthalmic grading of facial nerve palsy. This is a cross-sectional validation study. Two clinicians independently assessed and graded each patient on the same day, masked to each other's grading. Four parameters are assessed in the CADS scale: Cornea (0-3, ±a), static Asymmetry (0-2), Dynamic function (0-3), and Synkinesis (0-2). One clinician reassessed the patients and performed the grading again at a minimum time interval of 1 hour later. A weighted κ analysis was performed to determine inter- and intraobserver reliability using 95% bootstrapped bias-corrected and accelerated (BCa) confidence intervals (CIs). Thirty-three patients (27 women, mean age 51.7, range 23-80 years) with unilateral facial nerve palsy were graded. The overall interobserver reliability was 0.80 (95% BCa CI: 0.68-0.91) for cornea, 0.93 for resting asymmetry (95% BCa CI: 0.55-1.00), 0.80 for dynamic function (95% BCa CI: 0.50-0.96), and 0.88 (95% BCa CI: 0.71-0.96) for synkinesis. The overall intraobserver reliability was 0.93 for cornea (95% BCa CI: 0.83-0.98), 0.82 for resting asymmetry (95% BCa CI: 0.53-0.96), 0.92 for dynamic function (95% BCa CI: 0.72-1.00), and 0.98 for synkinesis (95% BCa CI: 0.84-1.00). The CADS grading scale demonstrates good interobserver reliability and very good intraobserver reliability. It incorporates all ophthalmic complications for facial nerve palsy and remains easy to use and refer to.

The effect of n-acetyl-cysteine on recovery of the facial nerve after crush injury.

ObjectiveFacial nerve dysfunction can vary in severity and recovery is dependent on the character of the injury. N‐acetyl‐cysteine prevents oxidative stress and cellular damage, and its use in the setting of nerve dysfunction from crush injury has not yet been established. In this study, rats with facial nerve crush injury will be treated with n‐acetyl‐cysteine or control and functional recovery and electrophysiologic outcome will be compared.Study DesignProspective, randomized animal studyMethodsTwenty‐four Wistar rats underwent unilateral facial nerve crush injury. Rats were implanted with a subcutaneous osmotic pump filled with saline (n = 12) or n‐acetyl‐cysteine 50 mg/kg/day (n = 12). Functional and electromyographic recovery was recorded at two and four weeks postoperatively.ResultsWhen compared to untreated rats, n‐acetyl‐cysteine treated rats had a greater electromyography amplitude recovery at 2 weeks with regard to eye blink (p=0.006) but not vibrissae function. At four weeks, the electromyography amplitude recovery of the vibrissae function was greater in n‐acetyl‐cysteine treated rats (P=0.001), but the amplitude recovery difference in eye blink was only marginally significant between groups (p=0.07). The functional score was higher in n‐acetyl‐cysteine‐treated rats than in untreated rats at all of the time points.ConclusionThis study demonstrated that n‐acetyl‐cysteine facilitated facial nerve recovery with improved functional and electromyography outcomes in the setting of crush injury.Level of EvidenceNA

Recovery in 61-Year-Old Male After Bell’s Palsy: A Case Report

Peripheral facial nerve palsy is the most common cranial nerve motor neuropathy. In majority of the cases, the cause is idiopathic and improvement of facial muscle function begins within first 3 weeks after onset of disease. This report presents a case of 61-year-old male patient who recourse to the emergency department with facial pain and asymmetry followed by peripheral unilateral facial nerve paralysis that there was no obvious cause. The patient eventually was discharged from hospital with an outpatient therapy of corticosteroid for 3 weeks, the regular courses of physical rehabilitation for the facial muscles weakness and recommendation to return for follow up in the future. Finally, after 4 months of treatment, the patient's facial weakness disappeared thoroughly without complication. In most cases, Bell’s palsy has a good prognosis and the patients will recover with outpatient therapy without complication.

Complete denture rehabilitation of a fully edentulous patient with unilateral facial nerve palsy: A case report

Complete denture rehabilitation of a fully edentulous patient with unilateral facial nerve palsy: A case report

Facial Nerve Recovery in KbDb and C1q Knockout Mice: A Role for Histocompatibility Complex 1.

Background:Understanding the mechanisms in nerve damage can lead to better outcomes for neuronal rehabilitation. The purpose of our study was to assess the effect of major histocompatibility complex I deficiency and inhibition of the classical complement pathway (C1q) on functional recovery and cell survival in the facial motor nucleus (FMN) after crush injury in adult and juvenile mice.Methods:A prospective blinded analysis of functional recovery and cell survival in the FMN after a unilateral facial nerve crush injury in juvenile and adult mice was undertaken between wild-type, C1q knockout (C1q−/−), and KbDb knockout (KbDb−/−) groups. Whisker function was quantified to assess functional recovery. Neuron counts were performed to determine neuron survival in the FMN after recovery.Results:After facial nerve injury, all adult wild-type mice fully recovered. Juvenile mice recovered incompletely corresponding to a greater neuron loss in the FMN of juveniles compared with adults. The C1q−/− juvenile and adult groups did not differ from wild type. The KbDb−/− adults demonstrated 50% recovery of whisker movement and decreased cell survival in FMN. The KbDb−/− juvenile group did not demonstrate any difference from control group.Conclusion:Histocompatibility complex I plays a role for neuroprotection and enhanced facial nerve recovery in adult mice. Inhibition of the classical complement pathway alone does not affect functional recovery or neuronal survival. The alternative and mannose binding pathways pose alternative means for activating the final components of the pathway that may lead to acute nerve damage.

Primary Hodgkin’s lymphoma of the middle ear: A rare cause of facial nerve palsy

Facial nerve palsy can occur whenever any part of the facial nerve is affected. It can be complete or partial, unilateral or bilateral and upper motor or lower motor neurone type. Common causes of unilateral lower motor neuron facial nerve palsy include trauma, infections of the middle ear, neoplasms of the parotid, iatrogenic and idiopathic. Hodgkin lymphoma typically presents with an asymptomatic lymphadenopathy, but associated symptoms include constitutional symptoms, intermittent fever, chest pain, or shortness of breath. We present an extremely rare case of facial nerve palsy secondary to primary classical nodular sclerosis Hodgkin’s lymphoma, arising from the left middle ear extending into the external auditory canal.

An unusual cause for facial nerve palsy: mucormycosis

Rhinocerebral mucormycosis is a rapidly fatal fungal infection affecting mostly uncontrolled diabetics. Multiple cranial nerves can be affected; however, palsy of the facial nerve has been rarely reported. Three uncontrolled diabetic patients presented to our outpatient department with features of rapidly progressive sinusitis and unilateral facial nerve palsy which on further evaluation was proven to be mucormycosis. Patients with uncontrolled diabetes and facial nerve palsy can have an underlying mucormycosis. This is important as early diagnosis and treatment is very prudent for a favorable treatment outcome in mucormycosis.

The effective stimulating pulse for restoration of blink function in unilateral facial nerve paralysis rabbits, verified by a simple FES system.

The trains of 200ms biphasic square pulses with the width of 9ms delivered at 50Hz were found to be the most suitable and effective mean as stimulation in FES system of restoring the blink function in unilateral facial nerve paralysis rabbit model. FES system is a reliable tool for these patients. Facial paralysis affects thousands of people every year. Many will have long term facial difficulties and the loss of the ability to blink the eye, which can lead to potential loss of the eye. Although many treatments exist, no one approach corrects all the deficits associated with the loss of orbicularis oculi function. FES is a means of providing movement in paralysed muscles to assist with practical activities and one possible way of restoring blink and other functions in these patients. Although some previous researches had investigated the effect of simple FES system on restoration of paralyzed facial muscles, there is still controversy about the appropriate details of the most effective stimulating pulses, such as the frequency, wave pattern and pulse width. Our aim is to find out the parameters of the most appropriate and effective stimulatin verify it by a simple FES system. 24 healthy adult male New Zealand white rabbits were accepted the surgery of right side facial nerve main trunk transaction under general anesthesia as the unilateral facial nerve paralysis models. The platinum tungsten alloy electrodes were implanted in orbicularis oculi muscle. The parameters of stimulus pulses were set to a 200ms biphasic pulse with different waveforms (square, sine and triangle), different frequencies (25, 50, 100Hz) and different widths from 1 to 9ms. Next, we set up a simple FES system to verify the previous results as the stimulus signal. We observed the movement of the both sides of eyelid when eye blink induced by different kinds of pulses. In all animals, the three kinds of waveforms pulse with frequency of 25Hz could not evoke the smooth blink movement. But the pulses with frequency of 50 and 100Hz can achieve this effect. The voltage threshold of the square pulse was lower than that of the sine pulse and triangle pulse. With the increase of pulse width from 1 to 9ms, the voltage threshold decreased gradually. The voltage threshold of the pulse with frequency of 100Hz was obviously lower than that of 50Hz. But the amount of total charge of the stimulation pulse of 100Hz was significantly more than that of 50Hz. In addition, when the FES system was turned on, the eye blink on the affected side with the stimulation pulses that were set by the previous step results was successfully aroused by the blink movement as a trigger on the contralateral.

Three-dimensional constructive interference in steady-state magnetic resonance imaging diagnosis of isolated unilateral facial nerve hypoplasia.

Three-dimensional constructive interference in steady-state magnetic resonance imaging diagnosis of isolated unilateral facial nerve hypoplasia.

Horner’s syndrome and unilateral facial paresis in a goat: a case report

A 4-year-old, female Pakistani goat was referred to the veterinary teaching hospital for first pregnancy check up. At the time of presentation, the case was apparently healthy. Incidentally, the clinicians noticed the abnormality in the left eye. A precise ophthalmic examination revealed ptosis, entropion, nictitating membrane prolapse, enophthalmos, and miosis in the left eye, whereas the eyelids and pupil were normal on the right side. The left eye lacked palpebral reflex, and the menace response seemed to be decreased. Pupillary light reflex was present in both eyes. The eyes did not show any evidence of trauma and irritation. The cornea appears to be healthy and transparent. On direct ophthalmoscopy, no gross abnormality was found in the eyeball. Unilateral facial palsy of the left side was prominently noticed, as the muzzle was deviated toward the contralateral side. A careful otoscopic examination revealed no evidence of otitis externa. Moreover, results of complete hematological and serum biochemical profiles were normal. In this goat, no other significant abnormalities were detected in physical examination. Detailed history delineated normal vision in both eyes, normal posture, and normal behavior. According to the clinical signs, a diagnosis of unilateral facial nerve paralysis as well as concurrent Horner’s syndrome was made. However, the exact etiology of these conditions remained unknown. The case was discharged without any treatment, and it was recommended to apply lubricating eye drops to prevent moisture from leaving the cornea.

Advances in Epidemiological Studies of Herpes Zoster

AbstractHerpes zoster (HZ) is a varicella zoster virus (VZV) that attacks locality of nerves and skin, resulting information of clusters of blisters on the skin connected unilateral facial nerve and accompanying apparent nerve pain. Incidence ranges from 3.2 to 4.2 per 1000 populations per year in the United States. Influence factors of HZ include age, infection history with VZV, vaccination history of varicella vaccine, reduced immunocompetence, and other diseases. Current domestic studies on HZ mainly focus on clinical reports of case treatment. This study reviews advances in foreign epidemiological studies of HZ.

Atypical and incomplete Kawasaki disease

Incomplete Kawasaki disease (KD) occurs in patients with fever lasting five or more days and with two or three of the classical findings (exanthema, conjunctivitis, changes in the extremities, erythema of oral mucosa and lips, cervical lymphadenopathy) [1]. It is difficult to define its real incidence. However the association between bilateral conjunctival bulbar injection and perineal erythema, with early desquamation in a patient with platelet count > 450.000 after 7 days of fever is indicative of incomplete KD. The definition “atypical KD” should be reserved for patients who have clinical manifestations such as renal impairment, unilateral peripheral facial nerve palsy, testicular swelling, pulmonary nodules and/or infiltrates, pleural effusions, diarrhea, vomiting and abdominal pain, acute surgical abdomen, hemophagocytic syndrome that generally are not seen in Kawasaki disease [1]. The “classical” clinical criteria have low sensitivity and specificity and therefore, other clinical and laboratory features may be helpful in establishing the diagnosis, especially for atypical or incomplete KD. In the last years many studies highlighted clinical and/or laboratory findings useful to obtain a precocious diagnosis in atypical and incomplete KD, because coronary artery lesions (CAL) occur in 15-25% of untreated KD and approximately in 5% of KD who received intravenous immunoglobulin (IVIG) treatment before 10 days of fever [2,3]. Relief of hyponatremia, hypoalbuminemia, increased D-Dimer are biochemical findings useful for diagnosis [4]. The association of more parameters, also considering AST, ALT, gamma-GT, leukocytes, percentage of neutrophils, platelet counts, CRP, ESR can be a help in the labyrinth of KD. In fact a precocious diagnosis and a timely treatment with IVIG significantly improve prognosis and reduce the risk of CAL. However the doubt of an atypical KD requests electrocardiography and echocardiography, to exclude CAL, pericardial effusion, valvular insufficiency, arrhythmia, prolonged PR interval, nonspecific ST and T wave changes in patients with FUO. Although aneurysms are rare before day 10 of fever, perivascular brightness, coronary arteries ectasia in the acute stage, indicate coronary arteritis before the aneurysms develop. incomplete KD should be considered in all children with unexplained fever for ≥5 days associated with 3 or less of the clinical criteria. incomplete KD is more common in young infants, who have a higher risk to develop CAL. Young infants may present with incomplete KD: echocardiography should be considered in infants younger than 6 months with fever for ≥7 days, laboratory evidence of systemic inflammation, no other explanation of the disease.