Incidence and risk factors of acute encephalopathy with biphasic seizures in febrile status epilepticus.

OBJECTIVE: To clarify the incidence and risk factors of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) in pediatric patients with febrile status epilepticus (FSE). METHODS: We retrospectively surveyed patients with FSE (≥20 min and ≥40 min) who were younger than 6 years by mailing a questionnaire to 1123 hospitals in Japan. The survey period was 2 years. We then collected clinical data on patients with prolonged febrile seizures (PFS) ≥40 min and those with AESD, and compared clinical data between the PFS and AESD groups. RESULTS: The response rate for the primary survey was 42.3%, and 28.0% of hospitals which had applicable cases responded in the secondary survey. The incidence of AESD was 4.3% in patients with FSE ≥20 min and 7.1% in those with FSE ≥40 min. In the second survey, a total of 548 patients had FSE ≥40 min (AESD group, n = 93; PFS group, n = 455). Univariate analysis revealed significant between-group differences in pH, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, creatine kinase, NH3, procalcitonin (PCT), uric acid, blood urea nitrogen, creatinine (Cr), and lactate. Multivariate analysis using stratified values showed that high PCT was an only risk factor for AESD. A prediction score of ≥3 was indicative of AESD, as determined using the following indexes: HCO3- < 20 mmol/L (1 point), Cl <100 mEq/L (1 point), Cr ≥0.35 mg/dL (1 point), glucose ≥200 mg/dL (1 point), and PCT ≥1.7 pg/mL (2 points). The scoring system had sensitivity of 84.2% and specificity of 81.0%. CONCLUSION: Incidence data and prediction scores for AESD will be useful for future intervention trials for AESD.

MeCP2_e2 partially compensates for lack of MeCP2_e1: A male case of Rett syndrome.

BACKGROUND: Rett syndrome (RTT) is a neurodevelopmental disorder that predominantly affects girls. Its causative gene is the X-linked MECP2 encoding the methyl-CpG-binding protein 2 (MeCP2). The gene comprises four exons and generates two isoforms, namely MECP2_e1 and MECP2_e2. However, it remains unclear whether both MeCP2 isoforms have similar function in the brain. METHODS: We report a case of a boy with typical RTT. Male cases with MECP2 variants have been considered inviable, but somatic mosaicism of the variants can cause RTT in males. Whole-exome sequencing was performed to search for the genetic background. RESULTS: A novel nonsense and mosaic variant was identified in exon 1 of MECP2, and the variant allele fraction (VAF) was 28%. Our patient had the same level of VAF as that in reported male cases with mosaic variants in MECP2 exon 3 or 4, but manifested RTT symptoms that were milder in severity compared to those in these patients. CONCLUSION: This is probably because the variants in MECP2 exon 3 or 4 disrupt both isoforms of MeCP2, whereas the variant in exon 1, as presented in this study, disrupts only MeCP2_e1 but not MeCP2_e2. Therefore, our findings indicate that MeCP2_e2 may partially compensate for a deficiency in MeCP2_e1.

Regional volumetric assessment of the brain in moderately preterm infants (30-35 gestational weeks) scanned at term-equivalent age on magnetic resonance imaging.

BACKGROUND: Early volume analyses of the infantile brain may help predict neurodevelopmental outcome. However, brain volumes are not well understood in moderately preterm infants at term-equivalent age (TEA). AIM: This study retrospectively investigated the relationship between regional brain volumes and infant gestational age (GA) at birth in moderately preterm infants (30-35weeks' GA) on magnetic resonance imaging (MRI) at TEA. METHODS: Forty infants scanned at TEA were enrolled. Regional brain volumes were estimated by manual segmentation on MRI, and their relationship with GA at birth was assessed. RESULTS: The regional volumes of the cerebral hemispheres and deep gray matter were larger (Spearman ρ=0.40, P=0.01, and Spearman ρ=0.48, P<0.01, respectively), and volumes of the lateral ventricles were smaller (Spearman ρ=-0.32, P=0.04) in infants born at a later GA. The volumes of the cerebral hemispheres of the infants born at 30weeks' GA were significantly smaller than those born at 33 and 35weeks' GA (P<0.05). No associations were found between the volume of the cerebellum and brainstem, and GA at birth (Spearman ρ=0.24, P=0.13, and Spearman ρ=0.24, P=0.14, respectively). CONCLUSIONS: The volumes of the cerebral hemispheres at TEA may be smaller in infants born at 30weeks' GA, whereas those of the cerebellum and brainstem may not be correlated with GA among moderately preterm infants.

[A child case of surfer's myelopathy].

We report a case of so-called "surfer's myelopathy" involving a 15-year-old girl. Preceding episodes of vaccination and infection were absent. She experienced back pain during a surfing class for beginners, followed by weakness in both legs, which progressed to paraperesis. Then, 2 days later, sensory disturbance and bladder/rectal disturbance also developed. Spinal MRI revealed a ischemic lesion compatible with anterior spinal cord syndrome. A combination of methylprednisolone pulse therapy and high-dose intravenous immunoglobulin therapy was effective leaving no residual dysfunction. The acute onset during the surfing class and characteristic MRI findings led to the diagnosis of surfer's myelopathy. Although rarely reported, warnings are warranted to prevent surfer's myelopathy and avoid the progressive deterioration of neurological dysfunction.

[A case of rapidly progressive glomerulonephritis with anti-glomerular basement membrane antibody in the course of MPO-ANCA positive interstitial pneumonia].

A 78-year-old man developed rapidly progressive glomerulonephritis (RPGN) in the course of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive UIP that had been found four years previously. When UIP was diagnosed, the MPO-ANCA titer was low and urine was negative for proteinuria and hematuria. On admission, his serum creatinine increased to 16.89 mg/dL and hemoglobin decreased to 5.2 g/dL. Urinalysis revealed that urinary protein excretion was 0.423 g/day and hematuria (30-40/HPF). The MPO-ANCA titer increased to 95.6 U/mL and anti-glomerular basement membrane (GBM) antibody titer elevated to 140 EU. Renal pathology revealed cellular crescents in 10 out of 11 glomeruli excluding two global sclerotic glomeruli. Immunofluorescence showed heavy linear deposits of IgG and C3 along the GBM. Treatments were begun after admission with hemodialysis and intravenous methylprednisolone pulse therapy, oral prednisolone at the dose 30 mg/day. Both MPO-ANCA and anti-GBM antibody were within the normal range after four months. However, the renal function was not restored despite treatment and he died of pulmonary infectious disease after six months from the onset of RPGN. Recently, many cases of RPGN with both MPO-ANCA and anti-GBM antibody have been reported. In this case, persistent UIP-associated MPO-ANCA appeared to have triggered RPGN by anti-GBM antibody.

Consistently high levels of exhaled nitric oxide in children with asthma.

BACKGROUND: Exhaled nitric oxide (eNO) levels in children are unstable because they are regulated by many potent factors. The purpose of the current study was to evaluate the reliability of eNO levels between a long interval and other lung functions in normal and asthmatic children. METHODS: Eighty-three elementary school children (aged 11-12 years; male : female, 39 : 44) participated in this study. Lung function, airway resistance and eNO levels were measured twice: the first measurement was in autumn 2007, and the second was one year later. RESULTS: There were 62 non-asthmatic control children (male : female, 31 : 31) and 21 asthmatic children (male : female, 8 : 13). In both the first and the second examination, the levels of eNO in children with asthma were higher than those in children without asthma. The parameters of lung function and the respiratory resistance in children without asthma showed a good correlation between the results of the first and second examinations. The eNO level in non-asthmatic children showed a good correlation between the two. On the other hand, the peripheral airway parameters of lung function and the respiratory resistance in children with asthma were not correlated between the first and the second examinations. The eNO level in these patients was well correlated between the two examinations. CONCLUSIONS: These data suggest that the eNO level showed good reproducibility in children with and without asthma. The eNO level is therefore considered to be a useful marker for reproducibly evaluating a subject's airway condition.

A prognostic value of neuron-specific enolase in cerebrospinal fluid of acute cerebellar ataxia.

We examined 16 patients with acute cerebellar ataxia (ACA) to determine whether clinical manifestations or laboratory findings could predict the prognosis of ACA. We divided the patients into two groups: a benign group of patients whose cerebellar symptoms completely disappeared within 21 days (9 patients), and a prolonged group of patients whose cerebellar symptoms persisted for more than 22 days (7 patients). The two groups were compared on the basis of demographic and clinical characteristics, and laboratory variables. The cerebellar symptoms did not differ significantly between the two groups, except in duration. The level of neuron-specific enolase (NSE) in the cerebrospinal fluid (CSF) of the prolonged group was significantly higher than that of the benign group (p < 0.01); other parameters, including protein and cell count in the CSF, were not significantly different. The results suggest that the NSE level in CSF is of prognostic value in ACA.

[Recurrent idiopathic cerebral infarction in a 5-year-old boy, with emphasis on the importance of platelet aggregation analysis for appropriate selection of anti-platelet drugs].

We present a 5-year-old boy with recurrent idiopathic cerebral infarction in which analysis of platelet hyperaggregability was useful in choosing appropriate anti-platelet drugs. The patient presented with gait disturbance at the age of 5 years and 1 month. Brain MRI demonstrated multiple infarctions in the right thalamus and left cerebellum. There were no apparent underlying diseases including hematological, cardiac and vascular abnormalities. He was diagnosed as idiopathic cerebral infarction. First, we administered ticlopidine and he remained stable with persistent mild intention tremor in the left upper extremity for 4 months. Then he developed the second stroke at the age of 5 years and 5 months, and multiple infarctions in the right celebellum and cerebellar vermis were demonstrated. On platelet aggregation analysis, adenosine diphosphate (ADP)-induced aggregation was inhibited, probably due to ticlopidine administration. Collagen- and epinephrine-induced platelet aggregation showed hyperaggregation, so we started to administer cilostazol, which inhibits only epinephrine-induced hyperaggregation. We also added aspirin, which inhibits collagen-induced hyperaggregation. The combination of anti-platelet drugs inhibited epinephrine-, collagen- and ADP-induced hyperaggregation in this patient. He has been stable on the triple combination of anti-platelet drugs without further episodes of cerebral infarction or transient ischemic attack for 4 years to date. Appropriate selection of anti-platelet therapy was achieved by the simple and repeatable platelet aggregation analyses, which must be considered even in pediatric patients with cerebral infarction.

[Feasibility of helical CT in the diagnosis and management of tracheo-innominate artery fistula and tracheo-innominate artery allovascularity after tracheostomy in patients with severe neurological disorders].

Tracheo-innominate artery fistula (TIF) is one of the most important complications of tracheostomy in that it is often life threatening. We experienced two cases with TIF and tracheo-innominate artery allovascularity, in which three-dimensional helical CT (3DCT) was useful in making the diagnosis of TIF and tracheo-innominate artery allovascularity. In one case, acute hemorrhage occurred during the procedure of routine tube exchange, and hemostasis was achieved by inflating the cuff of the tracheostomy tube. After TIF was confirmed by 3DCT, surgical ligation of the innominate artery was performed. In the other case, the recognition of a pulsating movement of the tracheostomy tube prompted us to perform 3DCT, which demonstrated a collateral branch from the innominate artery to the trachea. Laryngotracheal separation was performed to prevent hemorrhagic events in the future. For patients with severe neurological disorders, 3DCT has the advantage that only a short time is required to produce a digital image demonstrating the structure surrounding the trachea, which is necessary for the management of the patients' airway.

Intravenous lidocaine for status epilepticus during childhood.

The clinical efficacy of lidocaine for convulsive status epilepticus in 53 convulsive episodes was examined in 37 children (17 males, 20 females). Mean age of patients receiving lidocaine was 3 years 7 months (SD 3y 5mo). Lidocaine administration achieved control of status epilepticus in 19 of 53 convulsive episodes (35.8%). Seizures ceased within 5 minutes of lidocaine administration in all 19 patients who were responsive to the drug. Regarding aetiology of status epilepticus and types of seizures, there was no statistical difference in effectiveness. Mild decrease of oxygen saturation, monitored by pulse oximetry, was observed in one patient, which improved by oxygenation using a mask. Lidocaine is a useful anticonvulsive agent; however, the response rate to lidocaine appears to be quite low, as less than half of the seizures were effectively controlled by lidocaine. Favourable properties of the drug include prompt responses, less alteration of consciousness, and fewer adverse effects, including less respiratory depression.

[Choice and administration sequence of antiepileptic agents for status epilepticus and frequent seizures in children].

We investigated the sequence of the administration, the efficacy and the safety of antiepileptic drugs (AED) given intravenously for the treatment of status epilepticus and frequent seizures in children. Our institute has a recommended sequence of AED administration for treatment of status epilepticus: the first-line agent is diazepam (0.3 - 0.5 mg/kg administered intravenously, once or twice). The second-line drugs include midazolam (0.15 - 0.4 mg/kg intravenously, once or twice, and if necessary, followed by continuous infusion at 0.06 - 0.18 mg/kg/hour), lidocaine (1 - 2 mg/kg intravenously, once or twice, and if necessary, followed by continuous infusion at 2 - 4 mg/kg/hour) and phenytoin (10 - 20 mg/kg, infused slowly). For those patients who previously experienced a seizure which was refractory to diazepam but responsive to the second-line agent, it was recommended to use the second-line agent as a first-line agent. When seizures were refractory to the first and second-line agents, thiopental was administered (3 - 10 mg/kg intravenously, and if necessary, followed by continuous infusion at 2 -5 mg/kg/hour). The etiologies of 177 occasions of status epilepticus and frequent seizures were categorized into two groups:epilepsy (n = 95) and situation-related seizures (n = 82). Situation-related seizures included febrile seizures (n = 44), acute encephalopathy/encephalitis (n = 31) and benign infantile convulsions (n = 7). The ages of the patients ranged from 0.1 to 18.4 years (average +/- SD:3.69 +/- 3.15 years). Diazepam was administered as the first-line drug on 157 of 177 occasions (88.7%). On 116 occasions the second-line agents were administered. Midazolam and lidocaine were injected as the second-line agent on 54 (46.6%), and on 33 (28.4%) occasions, respectively, although both midazolam and lidocaine injections were off-label use for seizure control in Japan. Thiopental was used as the third to fifth-line agent. Effective ratios (effective occasions/total occasions) of each drug were the following: thiopental 19/21 (90.4%), midazolam 57/99 (57.6%), lidocaine 25/60 (41.7%), phenytoin 16/41 (39.0%), diazepam 59/164 (36.0%). Thiopental was statistically more effective than midazolam, lidocaine, diazepam or phenytoin (p < 0.01), and midazolam was statistically more effective than diazepam (p < 0.01) or phenytoin (p < 0.05). Administration of thiopental caused complications more frequently than the other agents (p < 0.01): The complications by thiopental were severe in some cases requiring intratracheal intubations and artificial ventilation. From the viewpoint of both efficacy and safety, midazolam should be recommended as one of the first-line agents for status epilepticus.

Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen's encephalitis and chronic progressive epilepsia partialis continua.

PURPOSE: To evaluate antibody-mediated and cytotoxic T cell-mediated pathogenicity that has been implicated as the autoimmune pathophysiological mechanism in Rasmussen's encephalitis. METHODS: We examined autoantibodies against the N-methyl-d-aspartate glutamate receptor (NMDA-type GluR) epsilon2 subunit and its epitopes in serum and CSF samples from 20 patients [five histologically proven (definitive) Rasmussen's encephalitis with epilepsia partialis continua (EPC), four definitive Rasmussen's encephalitis without EPC, and 11 clinical Rasmussen's encephalitis with EPC]. We examined 3H-thymidine uptake into lymphocytes after stimulation by GluRs. RESULTS: All nine definitive patients (five patients with EPC and four without EPC), and 10 of 11 clinical Rasmussen's encephalitis patients had the autoantibodies. In four patients, the autoantibodies were absent in early stage when epileptic seizures had already become frequent, and appeared subsequently. In two patients, the autoantibodies persisted in the serum after frontal lobe resection or functional hemispherectomy, although epileptic seizures were completely controlled. Autoantibodies to the C2 epitope predominated, while autoantibodies to the extracellular N epitope were rare. The mean 3H-thymidine uptake ratios (stimulation by GluRepsilon2-containing homogenates/stimulation by PHA) were significantly higher in definitive and clinical Rasmussen encephalitis patients than in controls. The mean 3H-thymidine uptake ratios (relative to PHA) were significantly higher for GluRepsilon2-containing homogenate than for control homogenate or GluRdelta2-containing homogenate. CONCLUSIONS: Autoantibodies against GluRepsilon2 may be one of the diagnostic markers for Rasmussen's encephalitis with and without EPC. Patients have activated T cells stimulated by GluRepsilon2 in peripheral blood circulation. We speculate that cellular autoimmunity and the subsequent humoral autoimmunity against GluRepsilon2 may contribute to the pathophysiological processes in Rasmussen's encephalitis.

[Efficacy of lidocaine on seizures by intravenous and intravenous-drip infusion].

We retrospectively studied the efficacy of intravenous and intravenous-drip infusion administration of lidocaine on seizures treated in Saitama Children's Medical Center during the period of 1997-2002. Thirty eight seizures of 29 children (12 boys and 17 girls, average age 41.4 months) were treated with lidocaine. The overall rate of effectiveness was 53%. The response to lidocaine appeared within 5 minutes in all episodes. Good response to lidocaine was noted, in 36% of patients with epilepsy, in 72% with CNS infection and in 80% with benign infantile convulsions; in 42% with generalized seizures and in 63% with partial seizures. Thus there was no significant difference in efficacy among the underlying disorders and seizure types. The adverse reaction of SpO2 decrease was found in one patient. We conclude that lidocaine can be used as a second-line, anti-convulsive drug in status epilepticus based on its prompt effectiveness and low risk of side effects.

[Bell's palsy in children: analysis of clinical findings and course].

To evaluate treatment of Bell's palsy (acute idiopathic peripheral facial nerve paralysis) of children, the authors analyzed 38 cases (18 females, 20 males) of Bell's palsy in children aged below 16 years old. The mean age of all cases was 6.8 +/- 6.2 years old. All cases resulted in complete recovery within 6 months. Clinical score of facial motor functions were adapted to 17 patients who were more than 5 years old. They were divided into two groups: early recovery group (clinical symptoms recovered within 3 months; 10 cases) and later recover group (over 3 months; 7 cases). Clinical scores evaluated in the first week from the onset were not significantly different. Steroid therapy was used for 9 patients of early group and 6 patient of later group. All patients of this study were recovered, thus we could not evaluate effect of steroid therapy for Bell's palsy in children. Use of steroid therapy for Bell's palsy needs more concretely administration. We consider how the region locates near to the center is an important prognostic factor.

[A case of chronic cerebellitis with anti-glutamate receptor delta 2 antibody].

A 1-year-8-month-old patient developed cerebellar ataxia following a prodromal infection. Despite initial diagnosis of acute cerebellar ataxia, his symptoms lasted for more than 30 days. High-dose intravenous immunoglobulin and steroid pulse therapy failed to ameliorate his cerebellar symptoms, which fluctuated in association with infections. At the age of 3 years and 8 months, he had mental retardation with cerebellar symptoms. Findings of MR imaging and single photon emission computed tomography were normal. Neuron-specific enolase of cerebrospinal fluid (CSF) ranged from 10.4-17.6 ng/ml, correlating with the cerebellar symptoms. Serum and CSF anti-glutamate receptor delta 2 antibodies were detected in the serum and CSF. We diagnosed him as having chronic cerebellitis associated with anti-glutamate receptor delta 2 antibody.

Long-term results of tonsillectomy as a treatment for IgA nephropathy.

IgA nephropathy (IgAN) is the most common form of chronic glomerulonephritis with IgA deposits present mainly in the mesangial areas. We performed a 10-year retrospective case-control study of 71 patients with IgAN to evaluate the long-term effects and prognostic factors associated with tonsillectomy. Forty-one patients (19 males and 22 females) underwent tonsillectomy (Group A) and 30 patients (13 males and 17 females) did not (Group B). These patients were followed for more than 10 years after renal biopsy. The average age at initial renal biopsy was 29.78 years in Group A and 33.0 years in Group B. The average follow-up period was 13 years and 3 months in Group A, and 12 years and 7 months in Group B. Glomerular damage demonstrated on renal biopsy was more extensive in Group A than in Group B. Prognosis after 10 years of follow-up was compared between the two groups. The clinical remission rate was 24.4% in Group A and 13.3% in Group B, the stable renal function rate was 82.9% in Group A and 70.0% in Group B, and the renal survival rate was 95.1% in Group A and 73.3% in Group B. The renal survival rate in Group A was significantly higher than that in Group B (p <0.05). Although evaluation of renal pathology based on renal biopsy was useful in predicting the long-term effects of tonsillectomy in IgAN patients, the results of tonsillar provocation tests were not.

Long-term follow-up study of West syndrome: Differences of outcome among symptomatic etiologies.

OBJECTIVES: To evaluate the outcome of West syndrome and to elucidate the differences in the outcome related to the timing of brain injury. STUDY DESIGN: Medical records of 60 patients who were followed regularly for more than seven years were reviewed. The following clinical features were assessed: onset, seizure evolution, electroencephalography and intelligence. Those variables were compared among five groups: cryptogenic, prenatal, preterm, term, and postnatal groups. RESULTS: The onset ages of the postnatal group were later than those of the others (P<.05). The relapse after adrenocorticotropic hormone therapy of the preterm group was the earliest among the groups (P<.05). Regarding encelphalography, the ratio of patients with focal discharges was higher in the postnatal group than in the prenatal group (P<.05). The ratios of patients in whom focal epilepsy developed were higher in the term and postnatal group than in the cryptogenic and prenatal group (P<.05). The term group showed similar characteristics to those of the postnatal group. Seven of the 60 had normal intelligence, including three girls with tuberous sclerosis. CONCLUSION: The diverse outcomes of West syndrome depending on etiology seemed to be related to the timing of brain injury and brain development.

[Midazolam treatment for status epilepticus of children].

We investigated the efficacy and safety of midazolam given intravenously for the treatment of status epilepticus in children. Patients received one to three bolus injections of midazolam (0.15 to 0.40 mg/kg) until seizures disappeared completely. In other patients, continuous infusion of midazolam (0.06 to 0.48 mg/kg/hr) followed a bolus of midazolam or other anti-epileptic agents. The dose was increased every 15 minutes until the seizures were brought under control. The etiology of 62 episodes of status epilepticus was epilepsy in 43, acute encephalopathy/encephalitis in 11, febrile seizures in 7 and hypoxic encephalopathy in 1. The age of the patients ranged from 0.2 to 18.4 years (average: 3.7 years). Bolus injections were administered in 53 episodes. The average loading dose was 0.35 +/- 0.22 mg/kg (range 0.15 to 0.90 mg/kg). Of the 42 episodes in which seizures disappeared, 13 required no further continuous infusions. Of the remaining 29 in which continuous infusion was done at the rate of 0.06 to 0.60 mg/kg/hr (mean 0.30 mg/kg/hr), 21 ended in cessation of the seizures. The duration of continuous infusions ranged from 4 to 288 hours (average 49.0 hours). In 6 episodes there were adverse effects:transient hypoxemia (5) and agitation during withdrawal (1). No intervention was needed except oxygenation by mask for less than 72 hours. Midazolam is effective and safe, and can be used as the first line drug in the treatment of status epilepticus in children.

[High-dose intravenous immunoglobulin therapy in a child case of Bickerstaff's brainstem encephalitis].

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.

[MRI findings and effectiveness of cyproheptadine in two patients with ophthalmoplegic migraine].

We reported the MRI findings and clinical course of two patients with ophthalmoplegic migraine. Both patients presented with unilateral oculomotor nerve palsy. Contrast enhanced MR imaging revealed unilateral enhancement and thickening of the oculomotor nerve in one patient. Prednisolone was effective in both patients, but only could transiently. On the other hand, cyproheptadine hydrochloride could completely prevent recurrent attacks of ophthalmoplegic migraine. Thus, MR imaging with of contrast enhancement is useful in the diagnosis of ophthalmoplegic migraine. Cyproheptadine hydrochloride is better than prednisolone to prevention recurrent attacks and to avoid adverse effects.