A placebo-controlled crossover trial of creatine in mitochondrial diseases.

To test the efficacy and safety of creatine (Cr) monohydrate in mitochondrial diseases, 16 patients with chronic progressive external ophthalmoplegia or mitochondrial myopathy were randomized in a crossover design to receive double-blind placebo or 20 g Cr/day for 4 weeks. Cr was well tolerated, but there were no significant effects with regard to exercise performance, eye movements, or activities of daily life. The power of this pilot study was limited and future multicenter trials are needed.

Patients with somatoform phobic postural vertigo: the more difficult the balance task, the better the balance performance.

The objective was to test, whether the increased body sway activity, shown in patients with phobic postural vertigo (PPV) in a previous posturographic study, impairs postural balance during demanding balance tasks. In 17 patients with PPV and 15 normal subjects body sway was analyzed for two standing positions on a foam rubber-padded posturographic platform with the eyes open or closed: (a) normal upright stance, (b) tandem stance. During normal upright stance patients showed an increase in body sway activity between 0.1 and 19 Hz and in sway path values for lateral and fore/aft directions. During the most difficult balance task, i.e. tandem stance with the eyes closed, body sway activity and sway path values did not differ between patients and controls. Objective balance skills were not impaired in patients with PPV during balance tasks at the limits of postural control.

["Inner perilymph fistula" of the anterior semicircular canal. A new disease picture with recurrent attacks of vertigo]. / "Innere Perilymphfistel" des anterioren Bogengangs. Ein neues Krankheitsbild mit rezidivierenden Schwindelattacken.

In 1998 Minor et al. described a new variant of perilymphatic fistula: the "superior canal dehiscence syndrome". This syndrome is clinically characterized by recurrent attacks of vertigo and oscillopsia induced by loud noises or stimuli that result in changes in intracranial or middle ear pressure. It is caused by a dehiscence of bone overlying the superior (anterior) semicircular canal. Due to this dehiscence, a third, mobile window (in addition to the round and oval windows) is formed, and changes in pressure are pathologically transduced to the anterior semicircular canal. Although the superior canal dehiscence syndrome is not a rare condition, no other cases have yet been reported. Therefore, we describe a typical patient who suffered for many years from recurrent attacks of vertigo and oscillopsia induced by coughing and Valsalva's maneuvers. High resolution temporal bone CT scan showed a defect in the bone overlying the left anterior semicircular canal. Three-dimensional eye movement recordings using the search coil technique and subsequent vector analysis demonstrated that the eye movements were induced by excitation of the left anterior semicircular canal. We conclude that superior canal dehiscence syndrome is an important differential diagnosis in patients suffering from symptoms of a perilymphatic fistula, especially since it can be successfully treated by "plugging" of the affected semicircular canal. Such patients are thus spared unnecessary surgery of the middle ear.

Disease latency in Creutzfeldt-Jakob disease via dural grafting: a case report.

The case of a 57-year old man is reported who had been operated upon for cerebellar angioblastoma more than 19 years previously. Having received a lyophilized dural patch he developed Creutzfeldt-Jakob disease of which he died 19 months later. This is the longest latency with dura-associated Creutzfeldt-Jakob disease reported so far.