No To Hattatsu最新文献

Arterial spin labeling (ASL) is a magnetic resonance imaging (MRI) technique that enables visualizing of cerebral blood flow without need of a contrast medium. In recent years, there have been reports from outside Japan related to ASL use in migraine attacks. We report two cases of acute confusional migraine (ACM) in children. At time of confusion, ASL imaging showed reduced blood flow: for the first patient, in both cerebral hemispheres from the occipital lobe through the parietal lobe to the temporal lobe; for the second patient, throughout the left cerebral hemisphere. T1-, T2-, diffusion-weighted images, and fluid attenuation inversion recovery (FLAIR) images indicated normal results. Subsequent ASL re-examinations for both cases showed recovery from reduced blood flow. In our view, ACM can be characterized by a reduction in blood flow not limited to the occipital lobe but across wide regions of the cerebral hemisphere. We consider ASL to be helpful in the difficult differentiation of ACM from other disturbances of consciousness, in addition to enabling repeated examinations without the risks associated with single-photon emission computed tomography (SPECT) concerning radiation exposure or with contrast MRI concerning contrast media use.

To evaluate the diagnostic value of SPECT (single photon emission computed tomography) brain blood flow imaging for patients with non-herpetic acute limbic encephalitis (NHALE). A retrospective review of three patients who had clinical symptoms compatible to NHALE and were positive for anti-N-methyl-d-aspartate-type glutamate receptor (GluRε2) antibody. The patients consisted of a 6-year-old female, a 10-year-old female and a 13-year-old male, all of whom had limbic symptoms and were anti-GluRε2 antibody-positive. In all cases, brain MRI failed to detect any abnormality, but SPECT brain blood flow imaging was able to detect blood flow changes. All three cases showed some abnormality in their brain waves, and one of them also developed epilepsy. SPECT brain blood flow imaging may therefore be helpful for diagnosing NHALE which can lead to the development of either epilepsy or cognitive impairment.

Objective: Anti-epileptic drugs, such as carbamazepine (CBZ) or phenytoin, may induce hypothyroidism in epilepsy patients. We assessed thyroid function of chronic patients with severe motor and intellectual disabilities (SMID) in our hospital. Methods: We examined thyroid-stimulating hormone (TSH), free thyroxine (fT4), and free thyronine (fT3) in 73 patients with SMID (47 men and 26 women, average age 48.4 years, range 30-68 years) without thyroid hormone supplement therapy. We determined the relationship between the thyroid function of patients taking the anti-epileptic drugs CBZ, valproate (VPA), and phenobarbital, other medications including anti-psychotic drugs, and treatments without anti-epileptic or anti-psychotic drugs. Results: TSH levels were not significantly different between the groups taking CBZ (CBZ+med), other anti-epileptic drugs or anti-psychotic drugs (CBZ-med), and only medications without anti-epileptic or anti-psychotic drugs (Non-med). The CBZ+med group had significantly lower fT4 levels than the CBZ-med or Non-med groups. There was a negative correlation between thyroid function level and the phenobarbital groups. TSH levels of the VPA+med group were significantly higher than VPA-med and Non-med group; fT3 and fT4 levels were not significantly different. Conclusions: Our results indicate that hypothyroidism may be present in patients with SMID taking anti-epileptic drugs. This suggests it is important to regularly measure thyroid function in patients with SMID taking anti-epileptic drugs, especially CBZ.

Objective: We assessed the root mean square (RMS) of angular acceleration and the RMS of an angular jerk as expressions of the transformation of spontaneous movements in early infancy. Methods: During 36-56 weeks post-menstrual age (PMA), 15 premature infants (6 male, 9 female; 36 weeks PMA>) were measured every 4 weeks. A three-dimensional motion analyzer (Fastrak system; Polhemus Inc.) was used to measure spontaneous movements of the upper right limb in the supine infants. Upper limb position data were used to calculate the RMS of angular acceleration and the RMS of angular jerk at the elbow. The calculated data were classified into three terms: 36th and 40th week PMA (termⅠ), the 44th and 48th week PMA (termⅡ), and the 52nd and 56th week PMA (termⅢ). The typical value was the mean value for each term. Results: The RMS of angular acceleration in termⅡ was significantly less than that of termⅠ (p<0.05). The RMS values of angular jerk in termⅡ and termⅢ were significantly less than that of termⅠ (p<0.05). Conclusion: The RMS of angular acceleration and the RMS of the angular jerk are useful for expressing changes in the strength of spontaneous movements of elbow extension-flexion movements.

We report the case of a 14-month-old girl with Dravet syndrome carrying a splice-site mutation of c. 1170+1G>A on neuronal sodium channel alpha 1 subunit (SCN1A). She had a history of recurrent febrile or afebrile seizures since 4 months of age and developed acute encephalopathy due to infection with human herpesvirus-6, which presented with high grade fever, severe consciousness disturbances, and prolonged and clustered seizures. Electroencephalography showed a generalized slow activity. Intensive treatments, including mechanical ventilation, continuous thiopental infusion, and high-dose steroid therapy were initiated, and she gradually recovered. During the recovery phase, choreoathetosis-like involuntary movements appeared on the face and limbs, which were treated successfully with haloperidol. MRI findings during the acute phase were normal; however, diffuse cerebral atrophy became evident during the recovery phase. Single photon emission computed tomography (SPECT) of the brain revealed decreased cerebral perfusion over bilateral frontal and temporal lobes; however, perfusion of the occipital lobes, basal ganglia, and cerebellum remained normal. The patient showed serious developmental regression at discharge, with the loss of head control and meaningful words. Patient’s clinical course and the findings of SPECT resembled those of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), with the exception of lack of reduced diffusion of the subcortical white matter on the acute phase MRI and the prolonged and severe clinical symptoms. It has been reported that patients with Dravet syndrome are prone to complications, including various types of acute encephalopathies. Therefore, clinicians should carefully manage prolonged febrile seizures in patients with Dravet syndrome.

We report the case of a patient born with extreme muscle hypotonia, respiratory failure, and slightly elevated serum levels of lactic acid. Histochemical examination and mitochondrial respiratory chain enzyme activities of a muscle biopsy specimen revealed reduced activities of complexes Ⅰ, Ⅲ, and Ⅳ, diagnostic of mitochondrial respiratory chain disorder. Hypertrophic cardiomyopathy developed as a complication and additional therapy was administered at 3 months after birth. He was able to be discharged to home on applied home mechanical ventilation with tracheotomy at 1 year old. The patient survived until 4 years and 10 months of age, upon which he died of bronchitis. Early-onset mitochondrial respiratory chain disorder shows very poor prognosis and long-term survival has not been reported. Prompt assessment of mitochondrial respiratory chain enzyme activities is necessary for the diagnosis of congenital nonspecific multiple-organ failure, and early intervention may achieve better prognosis for mitochondrial respiratory chain disorder.