No abstract

We describe an unusual form of facial myoclonus activated by speech in 3 patients with different underlying neurological diseases and present the electrophysiological investigations and results of structural and functional imaging. In 1 of 2 patients in whom jerk-locked electroencephalogram (EEG) back-averaging was done, a cortical potential clearly preceded the facial jerks. In the second patient, a cortical potential preceding the jerk was not certain. In the third patient, the resting EEG contained outbursts of symmetric, slower frequencies of indeterminate significance. An epileptiform disorder was suspected in this patient.

A 32-year-old woman with a 4-year history of multiple sclerosis presented with persistent clawing of the right hand. History revealed that she and five family members had lifelong symptoms of paradoxical myotonia (impaired relaxation of muscles following muscle contraction), exacerbated by cold. The family was diagnosed with paramyotonia congenita, based on neurophysiological and genetic studies. To our knowledge, this is the first report of an Australian family with paramyotonia congenita. (MJA 2009; 190: 334-336) Clinical recordA 32-year-old woman of European ancestry was referred to a movement disorder clinic for evaluation of mild persistent clawing of the right hand (Figure, A), which had developed over the past year. She had no weakness of the hand or other neurological signs.The patient had been diagnosed with relapsing remitting multiple sclerosis 4 years earlier, based on clinical features (typical exacerbations), characteristic white matter changes on magnetic resonance imaging scans, and oligoclonal bands restricted to the cerebrospinal fluid. Her condition was managed in a multiple sclerosis clinic. Before her referral, she was in good health between exacerbations of multiple sclerosis.

No abstract

Multimodal representation of peripersonal or near space has been demonstrated in the brain of the nonhuman primate through invasive electrophysiological experiments. Representation of peripersonal space in the human brain has been inferred from extinction experiments and functional imaging studies. We present a unique case of lower limb myoclonus in a patient with common variable immunodeficiency which is sensitive to visual stimuli in the peripersonal space and light touch. This case provides further evidence for near space representation in the human brain. We hypothesize that somatopically organized multimodal areas exist in the human brain which code for peripersonal space.