A 6-Month-Old Girl with Incontinentia Pigmenti Presenting as Status Epilepticus.

Incontinentia pigmenti (IP) is an uncommon neurocutaneous syndrome. Its initial diagnosis is based primarily on characteristic papulovesicular skin lesions and early-onset neonatal seizures. In contrast to typical early neurologic manifestations, we encountered a normally developed 6-month-old female patient with hyperpigmented whorls on her body. Following respiratory syncytial virus infection and fever, the patient exhibited status epilepticus. Brain magnetic resonance imaging studies of the patient were compatible with the findings of acute encephalopathy in IP. Genetic analysis showed an 11.7 kb deletion within the gene encoding inhibitor of kappa-B kinase gamma. The patient was treated with anticonvulsants and subsequently reached expected developmental milestones after discharge. These findings indicate that when a patient presents with status epilepticus, meticulous examination for skin lesions should be performed to determine whether the patient has a neurocutaneous syndrome, such as IP.

Signal intensity ratio on magnetic resonance imaging as a prognostic factor in patients with cervical compressive myelopathy.

Patients with intramedullary signal intensity (SI) changes have a poor prognosis after surgical decompression in cervical compressive myelopathy (CCM); however, some patients show no clear relationship between the SI and postsurgical prognosis. This discrepancy may be because no comprehensive and proper quantitative evaluation exists to assess SI on magnetic resonance imaging (MRI). The purpose of this study was prospectively to evaluate the correlation between the clinical features, neurological outcome of patients with CCM, and the quantitative assessment of SI changes preoperatively and postoperatively, and the correlation with SI severity.A total of 112 patients with CCM at 1 or 2 levels underwent anterior cervical discectomy and fusion. We quantitatively analyzed MR signal changes on T1-weighted MR images (T1WI), gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA) contrast-enhanced T1WI, and T2-weighted MR images (T2WI) using the signal intensity ratio (SIR). We evaluated the correlations between various variables and neurological outcome using the Japanese Orthopedic Association (JOA) scale, and the severity of SI change by grade (i.e., grade 0 ["none"], grade 1 ["light"], and grade 2 ["bright on T2WI"]).Significant differences between the 3 grades existed in symptom duration, preoperative JOA score, SIR on T2WI, and JOA recovery ratio. The JOA recovery ratio was negatively correlated with symptom duration and the SIR on T2WI, and positively correlated with the preoperative JOA score and cord compression ratio, but not with the SIR on T1WI and contrast-enhanced T1WI. On the postoperative 12-month follow-up MRI, the JOA recovery ratio and SIR on T2WI of the SI reversal patients were better than those of the nonreversal patients. On multiple regression analysis, the SIR on T2WI was the main significant prognostic factor of surgical outcome.The grading system on T2WI provided reliable predictive information for neurological outcome. Quantitative alterations in the SI on preoperative and postoperative T2WI, but not T1WI or contrast-enhanced T1WI, reflected the clinical features, surgical outcomes, and the correlation with SI severity. The patients with a longer duration of symptoms, lower cord compression ratio, severe myelopathy, intense signal change (i.e., grade 2) on the spinal cord, and an SIR greater than 1.55 had a poor recovery after a surgical operation.

Prognostic factors for surgical outcome in spinal cord injury associated with ossification of the posterior longitudinal ligament (OPLL).

BACKGROUND: Ossification of the posterior longitudinal ligament (OPLL) may increase the risk of spinal cord injury (SCI) with various neurological deficits after minor trauma. However, few studies have investigated the influence of OPLL on neurological outcome after acute cord injury. We examined whether severe spinal canal stenosis caused by OPLL affects neurological outcome after SCI based on intramedullary signal intensity (SI) changes on magnetic resonance imaging (MRI). METHODS: From June 2006 to July 2013, we treated 246 patients with cervical cord injury. Fifty-one (20.7%) patients had ventral cord compression due to OPLL without any bony fractures. Among them, 38 patients (34 men, mean age 62.7 years) underwent cervical laminoplasty (8) and cervical decompression and fixation (30). The neurologic assessments were performed in patients who had 1-year follow-up, and the mean follow-up period was 42.2 months. OPLL type, cause of injury, cervical sagittal angle, cervical spine stenosis, cord compression ratio (space available for the spinal cord (SAC)), and grade of intramedullary SI (grade 0, none; grade 1, light; grade 2, intense T2WI) were assessed. RESULTS: Mean American Spinal Injury Association (ASIA) motor score at admission was 38.4 ± 21.9 (range, 2-70) and improved to 67.7 ± 19.1 (range, 8-94) at last follow-up (p < 0.05). Mean recovery rate of the motor score was 55.8 ± 19.9%. Five patients had SI grade 0, 20 patients had SI grade 1, and 13 patients had SI grade 2. Among the variables tested, age, initial ASIA motor grade, intramedullary SI grade, and SAC were significantly related to neurological outcome. However, initial cervical alignment, canal diameter, length of SI, time interval between injury and operation, and OPLL type had no significant effect on neurological outcome. CONCLUSIONS: Preoperative neurological status, cord compression ratio, and SI grade are related to neurological outcome in patients with SCI associated with OPLL. The better the preoperative neurological status, the more favorable the neurological outcome after surgery. A higher SI grade on preoperative T2WI was negatively related to neurological outcome. Therefore, the severity of SI change, cord compression ratio, and preoperative neurological status can be regarded as significant prognostic factors in patients with SCI associated with OPLL.

Bilateral accessory inferior turbinates and secondary middle turbinates.

Bifid inferior nasal turbinates-two inferior turbinates with a single root observed in the nasal cavity-are an extremely rare anatomical variation, especially when they are observed bilaterally and exist with other variations. We report a case of bilateral bifid inferior nasal turbinates with bilateral secondary middle turbinates. We discuss the findings of this rare condition and suggest an appropriate term-accessory inferior turbinate-to replace bilateral inferior turbinate by reason of its embryology.

The relevance of intramedullary high signal intensity and gadolinium (Gd-DTPA) enhancement to the clinical outcome in cervical compressive myelopathy.

PURPOSE: We prospectively investigated whether high intramedullary SI and contrast [gadolinium-diethylene-triamine-pentaacetic acid (Gd-DTPA)] enhancement in magnetic resonance imaging (MRI) are associated with postoperative prognosis in cervical compressive myelopathy (CCM) patients. METHODS: Seventy-four patients with ventral cord compression at one or two levels underwent anterior cervical discectomy and fusion (ACDF) for CCM between March 2006 and June 2009. The mean follow-up period was 39.7 months (range, 12.7-55.7 months). The cervical cord compression ratio and clinical outcomes were measured using Japanese Orthopedic Association (JOA) scores for cervical myelopathy. Patients were classified into three groups based on the SI change in T2WI, T1-weighted images (T1WI), and contrast (Gd-DTPA) enhancement. RESULTS: The mean preoperative and postoperative JOA scores were 10.5 ± 2.9 and 15.0 ± 2.1 (P < 0.05), respectively. The mean recovery ratio of the JOA score was 70.9 ± 20.2%. There were statistically significant differences in postoperative JOA and recovery ratio among three groups. However, post-surgical neurological outcomes were not associated with age, symptom duration, preoperative JOA, and cord compression. CONCLUSIONS: We found that intramedullary SI change is a poor prognostic factor and the intramedullary contrast (Gd-DTPA) enhancement on preoperative MRI should be viewed as the worst predictor of surgical outcomes in cervical myelopathy. Contrast (Gd-DTPA) enhancement and postoperative MRI are useful for identifying the prognosis of patients with poor neurological recovery.

Intramedullary high signal intensity and neurological status as prognostic factors in cervical spondylotic myelopathy.

PURPOSE: The neurological outcome of cervical spondylotic myelopathy (CSM) may depend on multiple factors, including age, symptom duration, cord compression ratio, cervical curvature, canal stenosis, and factors related to magnetic resonance (MR) signal intensity (SI). Each factor may act independently or interactively with others. To clarify the factors in prognosis, we prospectively analyzed the outcomes of patients with myelopathy caused by soft disc herniation in correlation with magnetic resonance imaging (MRI) findings and other clinical parameters. MATERIALS AND METHODS: From June 2006 to July 2009, we performed surgical operations in 137 patients with CSM. Of these patients, 70 (51.1%), including 45 men and 25 women with ventral cord compression at one or two levels, underwent anterior cervical discectomy and fusion. The mean duration of follow-up was 32.7 months. We surveyed the cervical curvature index (CCI), canal stenosis (Torg-Pavlov ratio), cord compression ratio, the length of SI change on T2WI, and clinical outcome using the Japanese Orthopedic Association (JOA) score for cervical myelopathy. The MRI SI was evaluated by grade: grade 0, no change in signal intensity; grade 1, light signal change; and grade 2, bright signal change on the T2WI. Multifactorial effects were identified by regression analysis. RESULTS: The mean preoperative and postoperative JOA scores were 10.5 ± 2.9 and 14.9 ± 2.1, respectively (p < 0.05). The mean recovery rate based on the JOA score was 70.0 ± 20.1%. The respective preoperative JOA scores and recovery ratios(%) were 11.6 ± 2.3 and 81.5 ± 17.0% in 20 patients with SI grade 0; 10.8 ± 2.3 and 70.1 ± 17.3% in 25 patients with grade 1; and 9.2 ± 3.6 and 60.7 ± 20.9% in 25 patients with grade 2, respectively. Post-surgical neurological outcome showed no significant relationship to age, symptom duration, cervical alignment, stenosis, or cord compression. CONCLUSIONS: Among the variables tested, preoperative neurological status and intramedullary signal intensity were significantly related to neurological outcome. The better the preoperative neurological status was, the better the post-operative neurological outcome. The SI grade on the preoperative T2WI was negatively related to neurological outcome. Hence, the severity of SI change and preoperative neurological status emerged as significant prognostic factors in post-operative CSM.

Clinical and electroencephalographic features of infantile spasms associated with malformations of cortical development.

The aim of this study was to reveal the clinical and encephalographic (EEG) features of infantile spasms (IS) with malformations of cortical development (MCDs). The clinical features, EEG findings, neuroimaging studies and outcomes of various therapeutic modalities for 27 patients with IS and MCDs were reviewed. Background activities of EEG on the MCDs, i.e. asymmetric hypsarrhythmia, localized persistent polymorphic slowing, asymmetric slowing and diffuse fast activities, were shown in 22, 15, 9 and 2 patients, respectively. Partial epileptiform discharges such as localized paroxysmal fast activities, spindle-shaped fast activities and subclinical seizures were shown in 15, 8 and 10 patients, respectively, and the lateralized prominence of generalized paroxysmal fast activities and generalized sharp and wave discharges in 4 and 5 patients, respectively. MCDs were suspected in 5 patients, as revealed by EEG and/or functional neuroimagings without distinct magnetic resonance imaging lesions, and confirmed by pathologic findings. Of the 11 patients treated with surgical resection, 8 became seizure free. EEG features can be very useful in the identification of underlying cortical dysgenesis in patients with IS. However, in one patient who underwent epilepsy surgery in early infancy, we observed easy bleeding and difficult hemostasis from friable vascular and parenchymal tissues. Various EEG features can be very useful in screening underlying MCDs. In addition, epilepsy surgery can be an effective therapeutic modality in many patients with otherwise medically intractable IS with MCDs. However, surgical intervention in extremely young infants should be performed with caution.

Dynamic contrast-enhanced MR imaging of VX2 carcinomas after X-irradiation in rabbits: comparison of gadopentetate dimeglumine and a macromolecular contrast agent.

RATIONALE AND OBJECTIVES: To compare enhancement patterns of gadomer-17 with those of gadopentetate dimeglumine in VX2 carcinomas after irradiation on rabbits. METHODS: Twelve rabbits with VX2 carcinoma in the thigh underwent dynamic contrast-enhanced magnetic resonance (MR) imaging with gadopentetate dimeglumine and gadomer-17 at 24-hour intervals before (n = 12), 3 days (group 1, n = 12), 1 month (group 2, n = 8) and 2 months (group 3, n = 4) after 30 Gy irradiation. After taking postirradiation MR images, 4 rabbits were killed for histopathologic examination at each time interval. The enhancement characteristics in MR imaging and morphology of tumor vessels in histopathologic specimen were assessed. RESULTS: After gadopentetate dimeglumine injection, the enhancement characteristics were not different among tumors before and after irradiation (P > 0.05). For gadomer-17, the enhancement ratios decreased after irradiation. The shape of the curves for tumor enhancement before irradiation was significantly different from curves of group 1(P < 0.05). The specimens from group 3 showed sclerosis and wall thickening in arterioles. CONCLUSIONS: Dynamic contrast-enhanced MR imaging with a gadomer-17 reveals increased capillary permeability at an early phase after irradiation and chronic obliterating vasculopathy at delayed phase.