Chronic isolated hemifacial spasm as a manifestation of epilepsia partialis continua

Abstract

The objective of this case study was to describe the clinical and EEG/fMRI data of a case of isolated hemifacial spasms (HFS) due to epilepsia partialis continua in a 59-year-old man with abnormal hemifacial movements that disappeared during voluntary tasks, were absent during sleep, and responded to carbamazepine. His neurological examination was normal; EEG showed right inferior frontal epileptiform discharges. EEG-fMRI showed increased blood oxygenation level-dependent contrast in the right inferior and middle frontal gyri corresponding to the contralateral motor and premotor cortex responsible for facial movements (BA 44, 45, 45,9) with widespread BOLD signal deactivations suggestive of epileptic network involvement despite a very focal epileptogenic process. We hypothesize that the response of some cases of HFS to carbamazepine, a first-line treatment in the pre-botulinum toxin era, may have been due to its anti-epileptic effects rather than to modulation of facial nerve hyperexcitability.

Cortical and subcortical contributions to absence seizure onset examined with EEG/fMRI

“…During EEG acquisition, time marks generated by the scanner were inserted automatically in the data stream corresponding to the beginning of each functional image acquisition. As previously, EEG data were collected continuously at 10 kHz using a MRI-compatible system (MagLink by Neuroscan, division of Compumedics Ltd, El Paso, TX) with software that included an algorithm to subtract gradient and ballistocardiographic artifacts (Scan 4.3.18) (29, 30). After low-pass filtering with a cutoff frequency of 60 Hz, the EPI gradient artifacts induced by imaging were averaged over the first 9 TR periods and then subtracted from each epoch of the raw data following a method described previously (31).…”

Epilepsia partialis continua successfully treated with levetiracetam

“…In adults, EPC has been attributed to various localized or generalized lesions, including infections, vascular deficits, tumour and systemic diseases [1][2][3][4][5]. Therefore treatment of EPC should concentrate on the underlying cause when possible [6].…”

“…Efficacy of pharmacological anticonvulsive treatment decreases with increased duration of the EPC [3,6]. In a few cases of long lasting EPC, successful treatment with valproic acid or carbamazepine has been described, but so far there is no general recommendation for a drug of choice [2,5]. Apart from the underlying disease, patients with EPC are often disabled with respect to their activities of daily living due their epileptic seizures and myoclonic jerks, depending on the range of movements and parts of the body involved.…”

“…His liver cirrhosis and portal hypertension made a selection of benzodiazepines, valproic acid and carbamazepine less appropriate, although myoclonic discharges could have been potentially treated by several other drugs as well [1][2][3][4][5][6].…”

“…Epilepsia partialis continua (EPC) in adults has been attributed to various causes, including hereditary defects, infections (encephalitis and cerebral abscess), vascular (stroke, ICH, subdural hematoma (SDH), neoplastic (primary and metastatic tumours) and metabolic (dysglycemias) disorders [1][2][3][4][5][6]. However, anticonvulsive treatment may be unsuccessful in most of these conditions [2,3,5,6].…”