Video 1
Segment 1: Upon admission, video reveals frequent myoclonic jerks affecting the left side of the body (neck, trunk, arm, and leg), leading to recurrent falls and requiring support for ambulation. Segment 2 and 3: At the ten-month follow-up, the patient exhibits subtle myoclonic jerks in the left arm while seated, and demonstrates significant improvement in mobility with independent walking and no falls. Segment 4: Ten months post-treatment, the patient is seen dancing enthusiastically, although reduced movement on the left side of the body is still noticeable.
Figure 1
The brain MRI reveals white matter hyperintensities on T2 FLAIR imaging, primarily affecting the right occipital region, bilateral high frontal regions, and additional areas in the left frontal region.
Figure 2
(Upper panel) The baseline EEG demonstrates abnormal activity, characterized by periodic generalized polyspike and wave discharges of moderate amplitude (350–450 microV), recurring at regular 3-second intervals. (Lower panel) Follow-up EEG: Improved polyspike and wave discharges (100–150 microV) now occurring every 5 seconds, indicating reduced abnormal activity.
Table 1
Clinical, Imaging, Treatment, and Follow-Up Characteristics of Published SSPE Cases* Demonstrating Long Survival or Substantial Improvement.**
| REFERENCE | COUNTRY | AGE/SEX | MEASLES VACCINATION | CHILDHOOD MEASLES | DURATION OF ILLNESS BEFORE PRESENTATION | CLINICAL PRESENTATION | DISABILITY AT PRESENTATION | NEURO-IMAGING | BRAIN BIOPSY | TREATMENT GIVEN | COURSE OF ILLNESS | OUTCOME |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Valente et al. 2021 [2] | Italy | 17/M | NA | Measles infection at 2 months | 4 months | Seizures Progressive encephalopathy Retinitis Periodic myoclonus | She needed help while walking and changing posture | Periventricular parieto-occipital T2/FLAIR hyperintensity | NA | Antiepileptics Methisoprinol and ribavirin Later Ketogenic diet | After 1 year, she improved | She became independent Able to read and write |
| Sonoda et al. 2021 [3] | Japan | 10/M | NA | 13 months of age | 2 months | Myoclonus | NA | Periventricular T2/FLAIR hyperintensity Progressive cerebral atrophy | NA | Intraventricular interferon-α and ribavirin | 14 years of follow-up | Patient remained stable through follow up |
| Nathan et al. 2019 [4] | India | 8/M | Not vaccinated | At 2 years of age | NA | Myoclonus Repeated falls | Akinetic mute stage | Periventricular T2/FLAIR hyperintensity | NA | Antiepileptics Isoprinosine, ribavirin, and lamivudine Later Ketogenic diet | 11 months | Myoclonus stopped Started speaking EEG normalised |
| Kwak et al. 2019 [5] | South Korea | 13/M | Single dose of MMR vaccine | At 2 years of age | NA | Myoclonus Progressive encephalopathy | Akinetic mute stage | Normal | NA | Intraventricular interferon-α and inosiplex | 13 years | Bedridden but able to speak |
| Eroglu et al. 2008 [6] | Turkey | Out of 19 patients, 10 had survival beyond three years. These patients, mostly males aged 14 to 22, survived between 36 and 160 months. Treatments included isoprinosine, often with α-interferon, but all eventually experienced disease progression and succumbed. | ||||||||||
| Prashanth et al. 2006 [7] | India | SSPE generally has a progressive, fatal course, yet a minority of patients exhibit long-term stabilization or remission, surviving over 3 years. This study of 19 such cases reveals variable presentations, prolonged stabilization/remission phases, and occasional functional recovery, | ||||||||||
| Miyazaki et al. 2005 [8] | Japan | 8/M | NA | NA | Sudden | Myoclonus Progressive encephalopathy | NA | Progressive brain atrophy | NA | Intrathecal interferon-α | 13 years | Akinetic mute stage Initially greatly improved quality of life for 7–8 years |
| Kurata et al. 2004 [9] | Japan | 17/F | NA | NA | NA | Myoclonus Progressive encephalopathy | Walking difficulty | NA | NA | Intrathecal interferon-α and inosiplex | 6 months | Patient improved and was able to speak and walk |
| Risk and Risk 2003 [10] | USA | 6/F | NA | NA | NA | Repeated drop attacks Mental decline | Walking difficulty | Periventricular T2/FLAIR hyperintensity | NA | Antiepileptics only | 3 months | Seizures controlled Able to walk Started attending school |
| Yazaki et al. 2000 [11] | Japan | 24/F | Not vaccinated | NA | 8 years | Progressive parkinsonism | NA | Periventricular T2/FLAIR hyperintensity Cortical atrophy | Presence of the gene region of the fusion (F) protein of measles virus in CSF Brain biopsy = neuronal degeneration, but absence of inclusion bodies | Intraventricular interferon-α and inosiplex | 7 months | Her parkinsonism disappeared. |
| Santosh Kumar and Radhakrishnan 1998 [12] | India | 25/M | NA | Measles at 24 months | 4 months | Seizures Mental decline Myoclonus | Akinetic mute | NA | NA | NA | 8 years | After 10 months started improving and even after 8 years she was independent |
| Woelfle et al. 1996 [13] | Germany | 12/F | Not vaccinated | Measles at 24 months | 4 weeks | Mental decline Spastic gait leading to difficulty in walking Seizures | Severe disability | Periventricular T2/FLAIR hyperintensity | NA | Antiepileptic drugs | 4 years | Patient improved substantially and improvement remained sustained |
| Barraclough 1983 [14] | United kingdom | 15/M | NA | Measles at 15 months | NA | Seizures Mental decline Myoclonus | 2 years later akinetic mute | NA | Brain biopsy = gliosis, hypertrophied astrocytes, perivascular cuffing No inclusion bodies | NA | 5 years | Able to walk and speak |
| Risk et al. 1978 [15] | USA 5 cases from the Middle East | In a Middle Eastern study, 5% of SSPE patients showed significant long-term improvement, with some surviving beyond three years. These patients displayed typical clinical signs, elevated measles antibodies, and stable or improved conditions up to 11 years post-onset. | ||||||||||
[i] EEG = Electroencephalography; F = Female; FLAIR = Fluid-Attenuated Inversion Recovery; M = Male; NA = Not available; USA = United states of America.
*SSPE cases those were diagnosed by Dyken’s criteria.
**Long term improvement included more than 3 years of duration. Substantial Improvement/remission defined as improvement in disability score.