Cerebellar pial arteriovenous malformations presenting with medullary venous hypertension: imaging and endovascular treatment.

A patient in their 60s presented with headache and progressive lower extremity weakness over 1 week. Initial MRI was thought to represent venous hypertension secondary to a dural arteriovenous fistula. However, angiography revealed a cerebellar pial arteriovenous malformation with medullary venous hypertension. The imaging and endovascular treatment of this unusual case of a pial cerebellar arteriovenous malformation presenting in that manner is presented.

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis.

OBJECTIVE: To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS. METHODS: A double-blind, placebo-controlled, multicenter randomized clinical trial was conducted. Participants with ALS (n = 296) were randomized (2:1) to receive topiramate (maximum tolerated dose up to 800 mg/day) or placebo for 12 months. The primary outcome measure was the rate of change in upper extremity motor function as measured by the maximum voluntary isometric contraction (MVIC) strength of eight arm muscle groups. Secondary endpoints included safety and the rate of decline of forced vital capacity (FVC), grip strength, ALS functional rating scale (ALSFRS), and survival. RESULTS: Patients treated with topiramate showed a faster decrease in arm strength (33.3%) during 12 months (0.0997 vs 0.0748 unit decline/month, p = 0.012). Topiramate did not significantly alter the decline in FVC and ALSFRS or affect survival. Topiramate was associated with an increased frequency of anorexia, depression, diarrhea, ecchymosis, nausea, kidney calculus, paresthesia, taste perversion, thinking abnormalities, weight loss, and abnormal blood clotting (pulmonary embolism and deep venous thrombosis). CONCLUSIONS: At the dose studied, topiramate did not have a beneficial effect for patients with ALS. High-dose topiramate treatment was associated with a faster rate of decline in muscle strength as measured by MVIC and with an increased risk for several adverse events in patients with ALS. Given the lack of efficacy and large number of adverse effects, further studies of topiramate at a dose of 800 mg or maximum tolerated dose up to 800 mg/day are not warranted.

The role of the EMS safety officer.

One of the basic duties of an EMS leader is to ensure the well-being of his or her organization and its members. This can be done by fulfilling the duties of the safety officer. In so doing, the leader is, in effect, serving as risk manager, consultant, adviser and coach. In many cases, the EMS manager provides a member with the opportunity to fill these roles by delegating the responsibility and authority of serving as EMS safety officer. The EMS safety officer essentially has two separate functions: that of the ISO and that of the HSO. Typically, the ISO is another trained officer appointed at specific incidents, while the HSO is primarily an administrator. Regardless of which approach is taken, the bottom line is that safety is as important to EMS providers as it is to firefighters--something that is sometimes forgotten along the way.

Proximally evoked soleus H reflexes in the evaluation of axonal neuropathy.

It is sometimes difficult to differentiate physiologically distal axonopathy from proximal root or nerve disease. Standard soleus H reflexes evoked by distal tibial nerve stimulation at the popliteal fossa are often absent in both syndromes. In this study we distinguish the two by the presence or absence of soleus H reflexes evoked by proximal sciatic nerve stimulation at the gluteal fold. In 12 normal subjects maximum H reflex amplitudes evoked by distal tibial and proximal sciatic stimulation were essentially equal. In 12 patients with suspected distal polyneuropathy, proximal but not distal stimulation evoked H reflexes. By contrast, in 10 patients with lumbosacral root or proximal nerve disease, both proximal and distal stimulation failed to elicit H reflexes. In 2 patients with lower motor neuronopathy, low but comparable amplitude H reflexes were evoked at both sites. We conclude that the presence of a proximally evoked soleus H reflex may provide specific evidence of distal axonopathy when standard soleus H reflexes are absent.

Multifocal motor neuropathy with conduction block and Campylobacter jejuni.

We describe a patient with acute multifocal motor neuropathy with conduction block (MMNCB) and high titers of immunoglobulin G anti-GM1 antibodies after Campylobacter jejuni enteritis. Treatment with intravenous immune globulin led to rapid improvement with return of normal function by 6 weeks. This is the first report of C. jejuni enteritis preceding MMNCB.

The all ulnar motor hand without forearm anastomosis.

Anomalous ulnar innervation of intrinsic hand muscles, inferred in a number of clinical series, has been documented only rarely in electrophysiological studies. Using near-nerve and standard conduction studies we evaluated 2 cases with nearly exclusive ulnar innervation of hand muscles. There was no evidence of anomalous communication in the forearm. Digital sensory fibers were normally distributed in median and ulnar nerves. The anomalous motor innervation most likely results from palmar communication between ulnar and median branches (Riche-Cannieu anastomoses). The normal distribution of digital afferents suggests that such anastomoses are formed primarily by motor axons.

Retinotectal projection in reeler mutant mice: relationships among axon trajectories, arborization patterns and cytoarchitecture.

The distribution of axons in the midbrain and thalamus of homozygous reeler mutant mice is anomalous. The cytoarchitecture of these regions is normal. In the normal mouse SC there is a distinct SO in which fascicles of retinotectal axons pass caudally before terminating in the overlying SGS. In reeler, by contrast, fascicles of retinotectal axons are distributed through the entire thickness of SGS as well as through SO. There are also abnormalities of fiber pattern in the thalamus, most notably in the region of the dorsal nucleus of the LGd. Retinotectal axon trajectory and patterns of terminal arborization in reeler and normal animals were compared by single-fiber HRP axonography. In normal mice, two distinct morphological classes of retinotectal axons form focal terminal arborizations at different radial levels in the superficial layers of the SC. Class U axons are of relatively small diameter and terminate in upper portions of SGS. Class L1 axons are of larger diameter and form terminal arbors which are confined to SO and deeper regions of SGS. Axons of both classes ascend to their terminal zones from parent axons which course through SO. Similarly, in reeler mice axons of both large and small diameter can be distinguished. However, many axons of both classes pass caudally in anomalous fascicles distributed through the full thickness of SGS and descend to terminate. Other axons pass in normal fashion in SO and ascend to terminate in SGS. Regardless of their trajectories, the small axons terminate superficially in SGS while the thick axons terminate deeper in SGS and/or SO, as in normal mice. These findings suggest that the ingrowth of afferents and the formation of terminal arbors are regulated by different mechanisms and that fiber architecture and cytoarchitecture are regulated by different mechanisms. It is not known if the anomalous fiber pattern in reeler adults arises in development through a defect in initial patterns of axon fasciculation or from a failure of axon elimination.

Postnatal changes in arborization patterns of murine retinocollicular axons.

The growth and arborization of murine retinocollicular axons have been studied by means of HRP axon filling during postnatal development. Transformations in arborization patterns have been correlated with changes in synaptic density in the superficial collicular neuropil and with the formation of synapses by HRP-filled axons. At all postnatal ages axons of the optic projection are fasciculated and most follow a rostrocaudally aligned path. On the day of birth the axons course through both stratum griseum superficiale (SGS) and stratum opticum (SO); during the following 4 days the axon trunks disappear from SGS and are subsequently found only in SO. From postnatal day (P) 0 to P3, the majority continue far caudally in the colliculus, giving rise to small ascending collaterals at multiple points along their course. Ultimately, usually by P3, one or two collaterals begin to branch profusely and by P5 the majority of axons give rise to a focal terminal ascending arborization. The general configuration of most arborizations at P3 approximates that of the mature axon. However, the richness of terminal branching increases from P3 through the first 2 postnatal weeks. Synaptic density is relatively low in the first postnatal week, and no synapses involving HRP-filled optic axons were identified in this interval. Subsequently, after elaboration of definitive arbors has begun, synaptogenesis in the surrounding neuropil accelerates. Synaptic density in the upper SGS approximates adult values early in the third postnatal week. By this time synaptic junctions involving the terminal arborizations of optic axons are abundant.

The morphology of optic tract axons arborizing in the superior colliculus of the hamster.

Single axons innervating the superficial layers of the hamster's superior colliculus (SC) were visualized using an HRP-filling technique. Five types of axons were distinguished. Experiments involving the removal of retinal and/or cortical input showed that three of these axon types originated in the contralateral retina with the fourth type most likely originating in the visual cortex. The origin of the fifth type, a widely branched varicose axon, is apparently subcortical. The two major types of presumed retinotectal axons (types U and L1) project to the stratum griseum superficiale (SGS) in a bilaminar pattern. Type U axons take relatively direct paths from the layer of optic fibers to form dense terminal arbors in the upper half of the SGS. Terminal fields for type U fibers showed fairly consistent dimensions. Their rostrocaudal extent ranged from 90 micron to 190 micron, averaging about 120 micron. Type L1 axons were thicker than type U axons and terminated in deeper regions of the SGS and in the stratum opticum (SO). Single axons of this type often gave rise to multiple branches which took separate, circuitous paths to a common terminal field. Terminal fields for type L1 axons varied more in extent than did type U fields, but 58% of them had fields 90-150 micron in extent. Each of the axon types found can be related to previous studies of populations of tectal afferents. The two major types of retinofugal axons fit a scheme of parallel ascending pathways. The findings also have interesting implications for the study of axonal development.

A minute fraction of Syrian golden hamster retinal ganglion cells project bilaterally.

Bilaterally projecting retinal ganglion cells (BPRGCs) in the adult Syrian golden hamster were identified through the use of two retrogradely transported neuronal labels, horseradish peroxidase and Nuclear Yellow, placed separately in each optic tract. The distribution and size of doubly labeled retinal ganglion cells were characterized and their numbers were determined. Strict criteria were used to exclude artifactual doubly labeled cells. This work revealed that: (a) BPRGCs comprise less than 0.01% of the entire retinal ganglion cell population, averaging 7.4 (SD = 3) cells per retina; (b) BPRGCs are found primarily in the upper, peripheral retina and not along the vertical meridian or in the temporal crescent; and (c) BPRGCs correspond in size to ordinary retinal ganglion cells in their immediate vicinity, thus providing no evidence that they comprise a separate population of cells. Electrophysiological collision experiments were also performed, with stimulating electrodes in the two brachia of the superior colliculi and a recording electrode in one optic nerve. A collision effect was not detected, thus supporting the anatomical findings of rare bilateral branching of optic nerve axons. The occurrence of BPRGCs may reflect occasional ambiguities in the cues that guide axons through the chiasm.