Vol 20, No 3 (2025)

The article examines the specifics of applying the Early Start Denver Model (ESDM) for children with autism spectrum disorders in Russia. A survey of 24 ESDM specialists was conducted in the form of semi-structured interviews. The aim was to determine the features of Russian practice of using the ESDM and to compare it with evidence-based implementation algorithms, as well as to assess the specialists’ satisfaction with the model.

The results showed that most ESDM practitioners in Russia are specialists with prior experience in ABA therapy. The Russian practice of ESDM application generally differs from research-based protocols in terms of intervention intensity, parent involvement and training, as well as the development of individualized programs. The authors conclude that the current Russian practice requires further research to confirm its effectiveness. The survey also demonstrated high satisfaction of specialists with the ESDM.

Rett syndrome caused by MECP2 mutations is difficult for diagnostics of epileptic seizures, since contact with these patients is difficult, and the clinical manifestations of seizures are difficult to distinguish from various motor disorders and stereotypes. Currently, the genetic variants of Rett syndrome and approaches to therapy have been well studied, a classification of electroencephalogram changes has been developed. Nevertheless, epilepsy in Rett syndrome is characterized by pharmacoresistance. One of the reasons for the difficulties in the treatment of epilepsy is the overdiagnosis of epilepsy, as well as the difficulties in the differential diagnosis of epileptic seizures with stereotypical behavior and hyperkinesis. Videoelectroencephalographic monitoring plays a crucial role in the differential diagnosis of epileptic and non-epileptic paroxysmal conditions.

This review is based on the consensus statement of the International League Against Epilepsy’s pediatric panel on the diagnosis and management of patients with epilepsy and attention-deficit/hyperactivity disorder (ADHD). The document has been expanded and supplemented by later publications.

Its main provisions:

1. ADHD is 3–5 times more common in children with epilepsy than in the general population; the risk of developing epilepsy in people with ADHD is also increased. No gender differences were found in the manifestations of ADHD in boys and girls with epilepsy. Taking valproate during pregnancy increases the risk of developing ADHD in the child.
2. The presence of intellectual and developmental disabilities, as well as poor seizure control, increase the risk of developing ADHD.
3. Screening for ADHD should be performed in all children with epilepsy, beginning at age 6, when the diagnosis of epilepsy is established. Children should be retested annually for ADHD symptoms, including after a change in antiepileptic medication.
4. The International League Against Epilepsy recommends the Strengths and Difficulties Questionnaire for ADHD screening.
5. Behavioral problems occur more frequently with polytherapy than with monotherapy. A number of antiepileptic drugs (for example, valproate, benzodiazepines, etc.) can worsen cognitive and behavioral problems in patients with epilepsy and ADHD. Sulthiame has good cognitive tolerability and is approved for the treatment of hyperkinetic behavior and behavioral disorders in epilepsy, making it the drug of choice for patients with epilepsy and behavioral disorders, such as ADHD.

Hirayama disease, or monomelic amyotrophy, is a quite rare type of cervical flexion myelopathy that causes atrophy of the cervical spinal cord. Today, Hirayama disease is an insufficiently studied pathology that requires a thorough examination and differential diagnosis with other variants of myelopathy, which is especially important for excluding such a serious phenomenon as the cervical variant of amyotrophic lateral sclerosis. The presentation of this clinical case makes it possible to increase clinicians’ awareness of Hirayama disease in order to ensure early diagnosis and treatment.