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Comments on several articles related to neurology. Central visual field in homonymous hemianopia; Hemipshere-specific cognitive and motor changes after unilateral posteroventral pallidotomy.

The authors report a case of an infant girl with macrocephaly-cutis marmorata telangiectatica congenita (Macrocephaly-CMTC). This patient presented with developmental delay, mild subcostal retractions, and occasional apneic spells. An MRI demonstrated mild to moderate lateral ventricle hydrocephalus, left hemi-megalencephaly, and left cerebellar tonsillar herniation with full occlusion of the cisterna magna. Her foramen magnum was narrowed, measuring 17.5mm in transverse diameter. This value was significantly below the 50th percentile for age, which is 23.5mm. Together, these findings were suggestive of cervicomedullary cord compression, concerning for sudden death. The patient underwent posterior fossa decompression by suboccipital craniectomy and cervical laminectomy. Initially due to hypertrophy and paralysis of the left true and false vocal cords, endotracheal intubation was not achieved, requiring tracheostomy tube placement. To our knowledge this is the first report of apnea in a patient diagnosed with M-CMTC, likely due to cervicomedullary cord compression and perhaps exacerbated by unilateral laryngeal hypertrophy. M-CMTC is a newly-described hemi-hypertrophy syndrome affecting the neurodevelopment of affected children. The authors emphasize airway obstruction secondary to unilateral hypertrophy of the vocal cords in addition to brainstem compromise as a consideration for the etiology of apnea in M-CMTC patients presenting with signs and symptoms of cervicomedullary cord compression.

Visceral noxious stimulation induces central neuronal plasticity changes and suggests that the c-AMP-dependent protein kinase (PKA) signal transduction cascade contributes to long-term changes in nociceptive processing at the spinal cord level. Our previous studies reported the clinical neurosurgical interruption of post synaptic dorsal column neuron (PSDC) pathway by performing midline myelotomy effectively alleviating the intractable visceral pain in patients with severe pain. However, the intracellular cascade in PSDC neurons mediated by PKA nociceptive neurotransmission was not known. In this study, by using multiple experimental approaches, we investigated the role of PKA in nociceptive signaling in the spinal cord and PSDC neurons in a visceral pain model in rats with the intracolonic injection of mustard oil. We found that mustard oil injection elicited visceral pain that significantly changed exploratory behavior activity in rats in terms of decreased numbers of entries, traveled distance, active and rearing time, rearing activity and increased resting time when compared to that of rats receiving mineral oil injection. However, the intrathecal infusion of PKA inhibitor, H89 partially reversed the visceral pain-induced effects. Results from Western blot studies showed that mustard oil injection significantly induced the expression of PKA protein in the lumbosacral spinal cord. Immunofluorescent staining in pre-labeled PSDC neurons showed that mustard oil injection greatly induces the neuronal profile numbers. We also found that the intrathecal infusion of a PKA inhibitor, H89 significantly blocked the visceral pain-induced phosphorylation of c-AMP-responsive element binding (CREB) protein in spinal cord in rats. The results of our study suggest that the PKA signal transduction cascade may contribute to visceral nociceptive changes in spinal PSDC pathways.

Background: NF-1 is one of the most common autosomal-dominantly inherited genetic disorders with an incidence of approximately 1:3500. We report a case and review the literature to characterize spontaneous spinal AVF that occur in neurofibromatosis (NF-1)., Case Report: A 51-year-old woman presented with NF-1 and progressive radiculomyelopathy. Angiography revealed an AVF terminating in a giant intraspinal epidural varix extending paraspinally through the C3/4 neural foramen. Trapping of the AVF attempted 18 years earlier prevented endovascular access for embolization, and vigorous bleeding made direct surgical resection impossible. Therefore, as palliation, arterial feeding collaterals were occluded, and surgically exposed tortuous veins were packed with coils. Laminectomies and partial resection of the epidural varix resulted in subtotal occlusion with clinical improvement., Conclusion: The spinal AVF associated with NF-1 appears to show dominant venous drainage to the intraspinal extradural and paraspinal venous plexus without evidence of intradural drainage. The vertebral artery is typically the origin of the fistula. A giant venous varix and numerous collateral feeders to the vertebral artery may give an AVM-like appearance. Clinically, the fistulae produce a syndromic triad including symptoms of NF-1, progressive radiculomyelopathy, and a bruit. Treatment is direct attack on the fistula by either surgery or embolization. If, however, a direct approach cannot be chosen, occlusion of feeding vessels combined with laminectomies can result in long-term symptomatic improvement.

A menu-driven, problem-focused neurological assessment system was constructed and implemented after concerns at a six-hospital teaching center about the quality of nursing assessments for patients with neurological diagnoses were validated. This system enables the physician to guide the nurse's assessment by ordering specific neurological tests for each patient. Extensive staff training took place to develop competence with this system. This new system has resulted in positive changes for this facility. Nurses acknowledge greater comfort with performing neurological assessment; documentation of assessment has improved; and the assessments are becoming more individualized for each patient. This system resulted in a higher quality of neurological care for patients.