Probable dysimmune epilepsia partialis continua manifesting as epileptic moving toes syndrome: electroclinical features of a challenging case.

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. We describe a 22-year-old woman with EPC manifesting with isolated toe movements, prevalent over the left side and initially misdiagnosed as psychogenic, clinically almost indistinguishable from those observed in "painful legs and moving toes syndrome". The continuous involuntary movements with EMG correlates of twitches lasting <100 ms, the sharp waves over fronto-central regions on EEG, and the marked asymmetry in somatosensory evoked potentials with higher cortical amplitude over the right side following peripheral stimulation over the left foot confirmed the epileptic nature of the symptoms, leading to the diagnosis of EPC. The toe movements were markedly reduced following steroid therapy, whereas the infusion of immunoglobulins caused aseptic meningitis. Despite an extensive diagnostic work-up (including a search for antibodies for paraneoplastic and autoimmune encephalitis), an ultimate aetiological diagnosis was not reached, although the dramatic response to corticosteroids strongly supported an underlying dysimmune pathophysiology. Diagnosing EPC can be challenging, especially if movements are confined to a very small body region or strongly resemble movements encountered in other conditions. EEG-EMG monitoring should be performed in patients with continuous involuntary muscular jerks in order not to overlook a diagnosis of EPC. [Published with video sequences on www.epilepticdisorders.com].

Epileptic ictal strabismus: a case report and review of the literature.

Ictal strabismus, sometimes associated with epileptic nystagmus, is an extremely rare epileptic phenomenon, suggestive of cortical involvement in monocular eye movement control. We describe a patient with ictal disconjugate contraversive horizontal eye deviation of cortical origin as the main clinical feature of a focal seizure. A 17-year-old, previously healthy woman had a seizure characterized by initial rightward conjugate eye deviation, followed by convergent strabismus due to adduction of the right eye towards the nose without conjugate left eye abduction (esotropia), forced leftward head deviation with impaired awareness, and subsequent evolution into a bilateral tonic-clonic seizure. Postictal and interictal neurological status were unremarkable; more specifically, neuro-ophthalmological examination revealed no nystagmus or altered eye motility. Ictal EEG showed a rhythmic theta activity over the right posterior temporal region, involving fronto-central regions when strabismus appeared. MRI showed cortical dysplasia in the right temporal lobe. Due to the low spatial resolution of scalp EEG, we could not identify with precision the symptomatogenic zone underlying ictal strabismus. However, the concomitant appearance of rhythmic theta activity over the right fronto-central region and the leftward head version with MRI perfusion sequences, showing cerebral blood flow increase in the right frontal eye field area, suggest involvement of the right frontal lobe. [Published with video sequence on www.epilepticdisorders.com].

Delayed oxaliplatin-induced sensorimotor polyneuropathy.

BACKGROUND: To date, only a few cases have been reported that indicate that a delayed polyneuropathy may occur after chemotherapy with oxaliplatin. The clinical and electrophysiological manifestations of this delayed neurotoxicity have never been well documented. CASE REPORTS: Nerve conduction studies were performed in 4 patients who developed acute peripheral neuropathy several months after completion of oxaliplatin-containing chemotherapy. Sensory nerve conduction was abnormal in all patients. In 2 patients, the electrodiagnostic studies showed a mixed axonal and demyelinating sensorimotor polyneuropathy. CONCLUSIONS: Delayed polyneuropathy occurring after oxaliplatin-based chemotherapy can be confirmed by electrophysiological studies.

Trigemino-cervical reflex abnormalities in patients with migraine and cluster headache.

BACKGROUND: Head pain arises within the trigeminal nociceptive system. Current theories propose that the trigeminal system is intimately involved in the pathogenesis of migraine. Short-latency responses can be recorded in sternocleidomastoid muscles after stimulation of the trigeminal nerve (trigemino-cervical reflex). This brainstem reflex could be a suitable method to evaluate the trigeminal system in migraine and CH. OBJECTIVE: The aim of the present study was to further elucidate the pathophysiology of migraine and cluster headache (CH) with special reference to the involvement of the central trigeminal system in the different forms of primary headache. METHODS: The trigemino-cervical reflex was investigated in 15 healthy subjects, in 15 patients having migraine with aura, in 15 patients with migraine without aura, and in 10 patients with CH. RESULTS: Significant abnormalities were observed in a great number of patients with both types of migraine and CH during the headache attacks, but only in migraine patients during the interictal period. The alterations are bilateral in migraine, unilateral in CH. CONCLUSIONS: Our results further support the relevance of brainstem mechanisms in the pathogenesis of migraine rather than of CH. These data, taken together with that from experimental head pain and functional imaging studies, demonstrate that primary headache syndromes may be distinguished on a functional basis by areas of activation specific to the clinical syndrome.

Motor cortex excitability changes following a lesion in the posterior columns of the cervical spinal cord.

We used transcranial magnetic stimulation (TMS) to explore if an impairment of central sensory function produced by an isolated lesion in the cervical posterior white columns would change motor cortex excitability. Cortical silent period duration was prolonged when compared with the control subjects, while central motor conduction and motor thresholds were in the normal limits. We first demonstrate that the involvement of the ascending proprioceptive sensory pathways in spinal cord diseases may have direct consequences on the activity of intracortical inhibitory interneuronal circuits. These findings further elucidate the role of afferent inputs in motor cortex reorganisation.