Table 4.
Treatment effect on EEG in patients with RTT.
| Study | Sample (n: Age) | Treatment Schema | EEG Settings | EEG Effects | Other Treatment Effects |
|---|---|---|---|---|---|
| [57] | RTT 75% MECP2 (12: 2–11, 20, 30) |
Folinic acid: 2-year prospective randomized, double blinded, placebo-controlled crossover |
Video 16-channel EEG (10–20 system) |
Improvement: ▼ Seizure rate (only in 2 out of 8 patients who had seizures at baseline) ▼ Frequency of epileptiform discharges (only in 3 patients) Worsening: 3 patients developed seizure during treatment ▲ Frequency of epileptiform discharges (2 patients) |
N/A |
| [214] | RTT 100% MECP2 with drug-resistant epilepsy (8:8–18) |
levetiracetam (LEV) prospective, pragmatic, OLE: mean dose achieved was 44.84 mg/kg/day SD 18.02 |
EEG at baseline and 6-months after beginning of treatment |
Improvement: ▼ Seizure rate, background EEG, epileptiform abnormalities ▼ Frequency of epileptiform discharges (in 6 out of 8 patients) |
N/A |
| [215] | RTT 100% MECP2 post-regression (17:2–10) | rhIGF-1: 20 wk, double-blind cross over: 0.12 mg/kg/twice a day | 128 channels; 5–10 min calm recording when girls watched movie of their choice | = Frontal alpha asymmetry (FA) Worsening: ▲ Frontal delta power ▼ Beta relative power ▼Gamma relative power |
Worsening: ▲ The Kerr severity scale, ▲ ADAMS Depressed Mood subscale, ▲ Visual Analog Scale ▲Hyperventilation Improvement: ▼ ABC-C stereotypy ▲ CSBS-DP Social |
| [216] | RTT 100% MECP2 stage III (10: 3–11) RTT untreated (9: 2–12) |
rhIGF-1: 20–24 wk: 0.1 mg/kg/twice a day | 10 channels; 1–2 h EEG | = Frontal alpha asymmetry (FA) Improvement: ▲ Theta frequency ▼ Delta amplitude |
Improvement: ▼ International Scoring System: ISS), ▲ RSS: Rett Severity Score ▲ Endurance to RSS testing |
| [217] | RTT 100% MECP2 (6 out of 10:2–10) | rhIGF1: 4 wk MAD + 20 wk OLE: 0.120 mg/kg) | Frontal and parietal channels | Frontal alpha asymmetry (FA) (Pre-OLE: R > L with trend to reverse Post-OLE) |
Worsening: ▼ Behavioral subtotal (MBA) Improvement: ▼ Apnea index |
| [218] | RTT 100% MECP2 with epileptiform activity 33 (13 + 12 + 8 dose groups): 2–14.5) |
Dextromethorph
an:
25 wk OLE 3 dose groups: 0.25 mg/kg/day; 2.5 mg/kg/day; 5.0 mg/kg/day; |
Neuroscan EEG system; Overnight recordings |
=Epileptiform spikes and sharp waves during non-REM sleep Improvement: Seizure rate (parental report): low dose group (in all? small number of patients who had seizures before OLE n = 5/4/2 at each dose group) |
Improvement: ▲ Receptive Language (5.0 mg/kg/day vs 0.25 mg/kg/day) ▲ * Behavioral hyperactivity (2.5 mg/kg dose) |
| [219] | RTT 100% MECP2 (10:10–21) ambulatory | Glatiramer acetate (GA): 24 wk of 20 mg/day | A 21-channel 1-h video EEG (wakefulness (n = 10), drowsiness (n = 5), sleep (n = 3)) | =Background EEG abnormalities =Seizure rate Improvement: ▼ Frequency of epileptiform discharges (in all 4 patients who had them at baseline) |
Improvement: ▲ Gait velocity ▲ Memory Novelty Score % ▼ Breath holding index/% |
| [137] | RTT N/S (9:2–8, stage 3) 17 TD aged-matched |
Cerebrolyzin (CL): 20 days (2.0–2.5 mL daily, i.m.). | 16 channels: eyes open (n= 6) and eyes close (n= 5); >8.4-s epochs |
Improvement: ▲beta activity in the parietal region and occipital alpha in the 8–9 Hz narrow band ▼ central and frontal theta the after CL |
Improvement: Qualitative assessment by clinicians and parents ▲ the behavioral activity ▲ attention level ▲ motor functions ▲ non-verbal social communication |
| [220] | RTT (33: 21 + 13: 5–36) |
Visual discrimination training: Short-term, 30 min (STT: n = 13 + 21) Long-term, 5 days, (LTT: n = 21 vs. Control: n = 13) |
19 dry electrodes (wireless): visual discrimination task, at rest eyes open |
After STT: ▼ Alpha and Beta Brain Symmetry Index (shift from L > R into R > L) After LTT: =Brain Symmetry Index Improvement Theta relative power (frontal left and parietal) ▲ Beta relative power (frontal right and parietal left) |
After STT: No changes Improvement after LTT: ▲ Length of fixation (FL) ▼ Time before first fixation (TFF) |
| [221] | RTT (31: 13–35) non-sham tDCS (n = 18) vs. sham tDCS (n = 13) |
TDCS stimulation on Broca’s area together with linguistic training for 20 min/day over 10 days |
21 EEG channels |
▲ Frequency and Power of Alpha and Beta =Frequency and Power of Theta |
Improvement: ▲ Attention ▲ Language abilities (the number of phonemes produced) |
| [222] | RTT MECP2 mutations: C468G, R133C, R306C (3: 29, 30, 31) with chronic language impairments |
TDCS stimulation on Broca’s area together with linguistic training, case-study design for 20 min/day over 10 days |
19-channels EEG (20 min wit eyes closed) |
▲ Frequency and Power of Alpha, Beta and Theta |
Improvement: ▲ language abilities (the number of vowel/consonant sounds and words and the production and comprehension through discrimination) ▲ motor coordination (functional movements) |