Collagen abnormalities in the spinal cord from patients with amyotrophic lateral sclerosis

During the last 10 years, we have demonstrated morphological and biochemical abnormalities of skin extracellular matrices in amyotrophic lateral sclerosis (ALS). However, currently little is known concerning collagen of the spinal cord in ALS. We measured the amount of collagen and characterized collagen at light and electron microscopic levels in posterior funiculus, posterior half of lateral funiculus and anterior horn of cervical enlargement of the spinal cord obtained from ten patients with ALS, 11 patients with other neurologic diseases (control group A), and ten patients without neurologic ones (control group B). In posterior half of lateral funiculus and anterior horn, (1) by light microscopy, there was no significant difference in vessel wall area between ALS patients and control groups A and B; (2) ultrastructurally, collagen bundles were more fragmented and widely separated, and the fibrils were randomly oriented in the perivascular space of capillaries in ALS patients, which were not observed in any areas of control groups or in posterior funiculus of ALS patients; and (3) the collagen contents in ALS were significantly lower (P<0.001 and P<0.001, respectively) than those in control groups A and B. Fragmented and widely separated collagen bundles in the interstitial tissue surrounding capillaries and markedly decreased amount of collagen in posterior half of lateral funiculus and in anterior horn of ALS could be related to the degeneration of the upper and lower motor neurons in the spinal cord in ALS, that is, selective neuronal vulnerability in ALS.     

Development of the brainstem: assessment by MR imaging

The morphological development of the brainstem was studied by means of MR imaging. The subjects were 74 cases ranging in age from 4 months to 16 years, and 6 adult cases. The brainstem development was rapid until 4-6 years of age and thereafter it slowed down. That is the brainstem showed exponential growth (w', t', v and u). The relationship between brainstem growth and the cranium size was divided into 4 types as follows: 1) linear increase with development (s/T-O), 2) plateau (w/T-I and v/RTP-LTP), 3) down and up (u/RTM-LTM and z/RTM-LTM) and 4) exponential (t/T-P). In the values of v, z (the size of the brainstem in axial view) and t/T-P (the ratio of the midbrain and the cranium size in sagittal view), there were significant sex differences for cases of 10-16 years old. These values in male subjects were greater than those in female subjects (v, p less than 0.05, z, p less than 0.01, and t/T-P, p less than 0.05). That is the brainstem in male subjects was greater than that in female subjects.     

MR imaging of dural arteriovenous malformations with ocular signs

Summary Four patients with dural arteriovenous malformation (AVMs) draining into the cavernous sinus, who presented ophthalmic manifestations, were studied by magnetic resonance (MR) imaging. In all patients signal decrease in the involved cavernous sinus was demonstrated in coronal spinecho (SE) imaging. It is attributable to rapid venous flow in the sinus, and this high velocity signal loss is a fairly pathognomonic finding in this condition. We stress the validity of MR imaging in the primary diagnosis of dural AVMs with ophthalmic symptoms.     

Correlation between somatosensory evoked potentials and long loop reflexes--basic investigation and its application for multiple sclerosis

Somatosensory evoked potentials (SEPs) and long loop reflexes (LLRs) to the median nerve stimulation were investigated on 25 normal controls and 25 patients with multiple sclerosis (MS). Fifteen normal controls were also subjected to LLR study by the common peroneal nerve stimulation. The mean height were 159 +/- 8.2 cm in normal controls and 160 +/- 8.9 cm in MS, respectively. LLRs were obtained with 100% reproducibility in all cases. Upper limb LLRs were recorded from m. abductor pollicis brevis by trigger stimulation during isotonic contraction of the thumb, while lower limb LLRs were recorded from m. peroneus longus by trigger stimulation during isotonic eversion of the foot. The threshold of LLR was lower than that of short latency reflex (H-wave) with the mean latency of 40.4 +/- 1.5 ms. The height of subjects revealed an obvious positive correlation not only with the latency of LLR but also with N 20 of SEP, whereas central conduction time was not. Furthermore, a significant correlation was present between the latencies of LLR and N 20, showing a twofold gradient of LLR against N20. There was a significantly prolonged latency difference between H-wave and LLR of lower limb as compared with that of upper limb. When the stimulation site was changed from the wrist to the elbow, the latency difference between M-wave and H-wave shortened. This fact, therefore, appears to be against "resonance hypothesis" that LLR is set off according to the intrinsic mechanical oscillation given to the muscle concerned.(ABSTRACT TRUNCATED AT 250 WORDS)