Tsutomu Yoshimoto

Development of the Brainstem and Cerebellum in Autistic Patients.

Studies of magnetic resonance images have revealed morphological disorders of the brainstem and cerebellum in autistic children and adults. When we studied development of the brainstem and cerebellum in autistic patients, we found that although the brainstem and cerebellum significantly increased in size with age in both autistic patients and controls, these structures were significantly smaller in autistic patients than in controls. The speed of development of the pons, the cerebellar vermis I–V and the cerebellar vermis VI–VII was significantly more rapid in autistic patients than in the controls. However, the speed of development of the other brain structures in the posterior fossa did not differ between autistic patients and controls. The regression intercepts of the brainstem and cerebellum as well as those of their components were significantly smaller in autistic patients than in controls. Results suggest that brainstem and vermian abnormalities in autism were due to an early insult and hypoplasia rather than to a progressive degenerative process.

Reduced brainstem size in children with autism

Recently, structural brain abnormalities as well as functional abnormalities of the brainstem have been reported in autistic children. The authors undertook an analytic study of the brainstem in autistic children by means of magnetic resonance imaging (MRI). The MRI scans of 29 autistic children were compared with 15 control MRI scans. The autistic children were divided into two groups according to DQ (IQ) level: the DQ (IQ) greater than or equal to 80 group and the DQ (IQ) less than 80 group. The midbrain and pons were measured, and the ratio of the midbrain and pons sizes versus the cranium size were calculated. The brainstem size was found to be significantly smaller in the autistic group. In particular, the reduction in brainstem size tended to be greater in the low DQ (IQ) group when compared with the high DQ (IQ) one, though there was no significant difference (p less than 0.1). This suggests that the brainstem is anatomically altered in autistic children.

Differences in brain metabolites between patients with autism and mental retardation as detected by in vivo localized proton magnetic resonance spectroscopy.

We performed volume-selective proton magnetic resonance spectroscopy (1H-MRS) of the brain with a 1.5 T magnet in 28 patients with autism, and compared the results with those from 28 age-matched patients with unclassified mental retardation and 25 age-matched healthy children. Peaks for N-acetylaspartate, choline and creatine, but not lactate, were observed in each group on 1H-MRS. The N-acetylaspartate/choline ratio was lower in patients with mental retardation than in patients with autism and controls (P = .05, respectively). However, there were no differences in the N-acetylaspartate/ choline ratios between patients with autism and controls, and the N-acetylaspartate/creatine and choline/creatine ratios did not differ among the three groups. These results suggest that N-acetylaspartate is decreased in patients with mental retardation and that a disorder or dysfunction of neurons in the brain exists. There also appear to be differences in the brain lesions or dysfunctions found in patients with autism and mental retardation. (J Child Neurol 1997;12:91-96).

Reduced N-acetylaspartate in the brain observed on in vivo proton magnetic resonance spectroscopy in patients with mental retardation

Volume-selective proton magnetic resonance spectroscopy (1H-MRS) of the brain was performed with a 1.5T magnet in 28 patients with unclassified mental retardation (MR) and in 25 age-matched healthy children. Peaks of N-acetylaspartate (NAA), choline (Cho), and creatine (Cr), but not of lactate, were observed in both groups on 1H-MRS. In all our subjects of this age range, 1H-MRS revealed an increase with advancing age in the ratio of NAA/Cho (P = .0031), but no developmental change in the NAA/Cr and Cho/Cr ratios. The NAA/Cho ratio was lower in patients with MR than in controls (P = .0016). The NAA/Cr ratio tended to be lower in the MR group, and the Cho/Cr ratio did not differ between patients with MR and controls. These results suggest that in patients with MR, NAA decreases and a disorder and/or dysfunction of neurons in the brain exists.

Proton magnetic resonance spectroscopy of brain in congenital myotonic dystrophy

Volume selective proton magnetic resonance spectroscopy of brain was performed on a 1.5 T magnet in 5 patients with congenital muscular dystrophy and compared to the results in 46 healthy children and 1 healthy adult. Peaks of N-acetyl aspartate, choline, and creatine but not lactate, were observed in both groups on proton magnetic resonance spectroscopy. Spectroscopy of controls revealed an increase with advancing age in the ratio of N-acetyl aspartate/choline and N-acetyl aspartate/creatine and a decrease in the choline/creatine ratio. In patients with congenital myotonic dystrophy, the N-acetyl aspartate/choline ratio did not increase with advancing age, but the N-acetyl aspartate/creatine ratio did. The choline/creatine ratio decreased with advancing age, which matched the results of controls. At any age older than 4 years, the N-acetyl aspartate/choline and N-acetyl aspartate/creatine ratios were lower in patients with congenital myotonic dystrophy than in controls. The choline/creatine ratio did not differ between congenital myotonic dystrophy and controls. These results suggest that in patients with congenital myotonic dystrophy N-acetyl aspartate decreases and there exists a developmental disorder of neurons in brain.

Pseudosarcomatous myofibroblastic tumor of the urinary bladder with massive intraperitoneal hemorrhage in a child

Abstract A 3‐year‐old boy presented with persistent abdominal pain, hematuria and facial pallor with progressive anemia. A computed tomography scan showed a massive intraperitoneal hemorrhage and tumor mass in the bladder. Histological examinations of the resected tumor revealed findings of pseudosarcomatous myofibroblastic tumor (PMT). The clinico‐pathological features of this case warn us that PMT can cause acute abdomen syndrome with massive intraperitoneal hemorrhage.

Child-onset neuropathy associated with an anti-GM1 antibody

Usefulness of burr-hole subdural drainage for postmeningitic subdural empyema in infants: Report of two cases Yasuhiko Akiyama ‘I *, Masaharu Konishi ‘, Nobuyuki Maruyama ‘, Kouzo Moritake a, Yu-ichi Takusa b and Hiroshi Ozasa b (” Department of Neurosurgery, Shimane Medical Uniuersity, Izumo, Shimane, Japan; b Department of Pediatrics, Shimane Medical University, Izumo, Shimane, Japan) Subdural empyema is a known, yet infrequent, sequela of bacterial meningitis. Once subdural empyema occurs, the patients fail to respond to antibiotic therapy, so appropriate surgical treatment is needed for removal of the source of infection. We present two cases of subdural empyema secondary to bacterial meningitis in infants in which burr-hole drainage was effective. These cases are 4-month-old and 7-month-old boys who had subdural empyema secondary to Hemophilus influenzae meningitis. Though the meningitis was in remission, and while an appropriate antibiotic therapy had been continued, they developed recurrent fever and a computed tomography (CT) scan with contrast material showed peripheral enhancement of the bilateral subdural fluid collection, which the CT scan on admission had been revealed. As these findings suggested the occurrence of subdural empyema, burr-hole drainage was performed. Postoperatively there was resolution of their fever and they did well neurologically. Burr-hole drainage under ultrasonography, CT or MRI guidance is less invasive and it is possible to remove the subdural pus no less effectively than by craniotomy. It was concluded that burr-hole drainage should be chosen as an effective and safe method for surgical management in subdural empyema in infants.