Episodic encephalopathy due to an occult spinal vascular malformation complicated by superficial siderosis.

Superficial siderosis (SS) of the central nervous system is a rare condition caused by chronic subarachnoid hemorrhage. Clinical manifestations typically include sensorineural hearing loss and cerebellar ataxia. Recurrent episodic encephalopathy in the setting of SS has not been reported. We describe a unique case of SS in a 67-year-old man with an 8-year history of episodic encephalopathy associated with headache and vomiting. The patient also had a history of progressive dementia, ataxia, and myelopathy. A diagnosis of superficial siderosis was made after magnetic resonance gradient-echo images showed diffuse hemosiderin staining over the cerebellum and cerebral convexities. No intracerebral source of hemorrhage was identified. The patient therefore underwent gadolinium-enhanced spinal MRI which suggested a possible vascular malformation. A therapeutic laminectomy subsequently confirmed an arteriovenous fistula which was resected. In SS, there are often long delays between symptom onset and definitive diagnosis. Early identification is facilitated by magnetic resonance imaging with gradient-echo sequences. When no source of hemorrhage is identified intracranially, then total spinal cord imaging is indicated to assess for an occult source of hemorrhage as occurred in our case.

Disorders of the trigeminal system.

The management of patients with trigeminal system dysfunction requires an understanding of the system's complex anatomy, which extends from peripheral nerve endings, through the skull base, cavernous sinus (V1, V2 only), and trigeminal ganglion, to the intraaxial nuclei, tracts, and cerebral cortex. The differential diagnosis is broad. Seemingly minor facial sensory loss may indicate an underlying malignancy (as in numb-chin syndrome). Painful syndromes of the trigeminal nerve are numerous and require careful categorization. Understanding trigeminal system anatomy and the appropriate use of imaging and electrodiagnostics should aid in the diagnosis and treatment of these disorders.

Minimally invasive tongue base surgery for obstructive sleep apnoea.

Moderate to severe obstructive sleep apnoea is usually associated with multiple levels of pharyngeal airway collapse, including tongue base obstruction. A new technique has recently been introduced that improves the nocturnal retro-lingual airway. This study was a prospective, non-randomized single-institution evaluation of a recently introduced surgical technique. Nineteen consecutive patients with previously untreated moderate to severe obstructive sleep apnoea underwent tongue suspension using the Respose system and concomitant palatopharyngoplasty (multilevel pharyngeal surgery). The patient demographics and treatment outcomes were prospectively collected and retrospectively analysed. There were 16 men and three women, with a mean (+/-SD) age of 44.9 years (+/- 14.2) and a mean pre-operative apnoea-hypopnoea index (AHI) of 42.8 +/- 24.8. Twelve of the 19 patients (63.2 per cent) have had post-operative polysomnography; eight of these 12 (67 per cent) met the standard criteria for surgical response. Among these eight patients, the AHI improved from 32.4 to 14.4 (p<0.01); the individual scores fell by a mean of 51.7 per cent. The apnoea index improved from 7.4 to 0.9 (p<0.01), with the individual scores falling by a mean of 81.4 per cent. There was anatomic and radiographic evidence of multilevel upper airway enlargement. Notably, the body mass index remained essentially unchanged (31.5 +/- 7.2 to 31.2 +/- 7.6, p>0.5). Subjectively, the mean Epworth sleepiness scale score fell from 11.0 +/- 5.4 to 5.4 +/- 3.8 (p<0.005). Four patients suffered transient velopharyngeal insufficiency, and two patients complained of limited anterior excursion of the tongue. There were no serious, long-term complications. The tongue suspension procedure represents a minimally invasive technique for improving the nocturnal retro-lingual airway in patients with obstructive sleep apnoea. It is easily performed by otolaryngologists, distinguishing it from other established techniques designed to address tongue base obstruction.