6. Prognosis

Chien et al. have stopped erratic myoclonus and suppressive-burst pattern on EEG in a mixed form of EME + MMPSI using dextromethorphan 20 mg/kg [29]. There are different opinions about usefulness of ketogenic diet in MMPSI. So, François et al. proclaimed that seizures in MMPSI are also resistant to ketogenic diet [33]. But specialists from Children's Neuroscience Centre of Royal Children's Hospital (Parkville, Victoria, Australia) published data about efficacy of the ketogenic

Surgical treatment of MMPSI is unreasonable because of diffuse nature of brain

callosotomy may be offered as a palliative intervention; however, there is no such

A group of scientists from the Pediatric Neurology Department of Azienda Ospedaliera Universitaria (Ancona, Italy) have published about positive experience of vagus nerve stimulation (VNS therapy) in three infants with pharmacoresistant

Our cases confirmed that MMPSI are resistant to antiepileptic therapy. Monotherapy had no significant effect in all patients. All patients with MMPSI failed to relieve from epileptic seizures. In 14 MMPSI cases, antiepileptic therapy was completely ineffective (56%), reduction of seizures >50% was observed in seven patients (28%), and only in six patients decrease of seizures was >75% (17.16%). Relatively effective combinations of antiepileptic drugs included

valproates with barbiturates (phenobarbital and hexamidine) and benzodiazepines. Clobazam 1 mg/kg was most effective among benzodiazepine groups. In two patients positive effect was observed with combination of levetiracetam, and in one case – with combination of benzodiazepine and topiramate. Phenytoin in two cases caused moderate positive effect with "escape effect." In one patient, frequency of seizures decreased during treatment with potassium bromide (50 mg/kg) but with side effects in the form of hypersomnia. High doses of vitamin B6 in two cases were

Ethosuximide, rufinamide, carbamazepine, and oxcarbazepine have no substantial positive effect. In one case, carbamazepine in cryptogenic focal frontal epilepsy with temporary positive effect caused subsequent aggravation of seizures with appearance of additional foci with clinical and electroencephalographic transfor-

Hormone therapy caused only a temporary moderate positive effect in eight

For emergent relief of SE of hemiconvulsive and secondary generalized tonic-clonic seizures in 15 cases of MMPSI, benzodiazepines (relanium and midazolam) had only a temporary effect in eight or were completely ineffective

Positive effect in SE in MMPSI was observed with sodium oxybate administration at a dose of 100–150 mg/kg, 400 mg/min. This was done in seven cases of hemiconvulsive (n = 3) and secondary generalized tonic-clonic SE (n = 4) resistant to benzodiazepines with a temporary regression (six cases) or a decrease of clinical-

In three patients with MMPSI, intravenous valproates caused significant positive effect in relieving SE, especially in cases of tonic-autonomic seizures with episodes of apnea, with aggravation during treatment with benzodiazepines [36]. The recommended dose was 25 mg/kg intravenous over 5 min with the following main-

Sodium thiopental (4 mg/kg for 2 min and then infusion of 0.2 mg/kg per minute) is the last chance to stop drug-resistant SE but caused death in one girl due

cases and was completely ineffective at other cases.

diet in children with this pharmacoresistant form of epilepsy [34].

experience in this form of epilepsy.

Epilepsy - Advances in Diagnosis and Therapy

MMPSI [35].

moderately positive.

mation into MMPSI.

in seven cases.

124

EEG paroxysmal events (one case).

tenance infusion – 2 mg/kg/h.

to central inhibition of cardiac activity.

damage and lack of clear local structural defect [3]. Theoretically, anterior

MMPSI is a form of epilepsy with poor prognosis. Within a few months after disease onset, frequency and duration of seizures increase up to the serial seizures and status epilepticus. A number of patients die in the first year of life due to multiple prolonged epileptic seizures, development of respiratory distress syndrome, and decorticate rigidity [30]. Based on the generalized clinical observations, mortality in this syndrome is 28% [3]. The results obtained by Marsh et al. are prognostically more favorable: during the 7-year follow-up, all six patients survived; however, psychomotor retardation with severe muscular hypotonia persisted in three of them, and only one patient reached seizure control for a long time [25].

Mortality at personal observed cases was 25.7% (n = 9); however, the expected mortality is higher due to short follow-up (1 year) in more than half of these patients. The oldest of the survived patients with MMPSI is 9 years old; there is gross delay of psychomotor development with unformed verticalization skills, absence of voice activity, spastic tetraparesis, and multiple focal asymmetric tonic, versive, pharyngo-oral, and dialeptic seizures.

Follow-up of patients with MMPSI allowed distinguishing the following subpopulations:

