Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
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Case Reports
Hypoventilation in glycine-receptor antibody related progressive encephalomyelitis, rigidity and myoclonus.
Glycine receptor (GlyR) antibodies have been identified in patients with rigidity and hyperekplexia, but the clinical phenotype associated with these antibodies has not been fully elucidated. The clinical features in two additional patients with GlyR antibodies are described. A 55-year-old man presented with stimulus-induced hyperekplexia and rigidity in the lower limbs and trunk. ⋯ She responded to benzodiazepines and has remained in remission. The clinical picture associated with GlyR antibodies includes autonomic dysfunction, cardiac arrhythmias and hypoventilation. It is important to recognise these serious complications early to limit mortality from this treatable condition.
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Endoscopic extradural anterior clinoidectomy and optic nerve decompression through a pterional port.
Since the first description of the intradural removal of the anterior clinoid process, numerous refinements and modifications have been proposed to simplify and enhance the safety of the technique. The growing use of endoscopes in endonasal and transcranial approaches has changed the traditional management of many skull base lesions. We describe an endoscopic extradural anterior clinoidectomy and optic nerve decompression through a minimally invasive pterional port. ⋯ An extradural approach helped minimize complications associated with infraction of the subdural space and allowed for the maintenance of visibility while drilling with continuous irrigation. Adequate 270° bone decompression of the optic canal was achieved in all specimens. Endoscopic extradural anterior clinoidectomy and optic nerve decompression is feasible through a single minimally invasive pterional port.
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Case Reports
Vertebral artery dissection after neck extension in an adult patient with Klippel-Feil syndrome.
The association between Klippel-Feil syndrome and vertebral artery dissection is quite rare. We report an adult patient with vertebral artery dissection and Klippel-Feil syndrome, to our knowledge only the third reported case of its kind. A 45-year-old woman with a known history of Klippel-Feil syndrome presented with occipital head and neck pain following forced neck extension. ⋯ While cervical fusion, as seen in Klippel-Feil syndrome, has previously been shown to cause neurologic injury secondary to hypermobility, the association with vertebral artery dissection is incredibly rare. We hypothesize that this hypermobility places abnormal shear force on the vessel, causing intimal injury and dissection. Patients with seemingly spontaneous vertebral artery dissection may benefit from cervical spine radiography, and this predisposition to cerebrovascular injury strongly suggests further evaluation of vascular injury following trauma in patients with Klippel-Feil syndrome or other cervical fusion as clinically warranted.
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Review Case Reports
Valproate induced hyperammonemic encephalopathy successfully treated with levocarnitine.
Valproate-induced hyperammonemic encephalopathy is an unusual but serious adverse effect that is usually characterized by the acute onset of impaired consciousness, focal neurological symptoms and increased seizure frequency. It has been reported to occur at therapeutic valproate levels. We report a patient who developed valproate-induced hyperammonemic encephalopathy after a short treatment with valproate and was successfully treated with levocarnitine. We discuss this patient and review the literature regarding the use of levocarnitine in similar patients.
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Cerebrovascular anastomosis (for example in the management of Moyamoya disease or complex aneurysms) is a rarely performed but essential procedure in neurosurgery. Because of the complexity of this technique and the infrequent clinical opportunities to maintain skills relevant to this surgery, laboratory training is important to develop a consistent and competent performance of cerebrovascular anastomosis. We reviewed the literature pertaining to the training practices surrounding cerebrovascular anastomosis in order to understand the ways in which trainees should best develop these skills. ⋯ After gaining sufficient dexterity, the trainee will be able to practice using biological materials followed by high fidelity models prior to actual surgery. Unfortunately, the effectiveness of each model has generally, to our knowledge, only been judged subjectively. Objective quantification methods are necessary to accelerate the acquisition of competence.