Table 1
Medical Investigations
| Blood Test | Result | Reference Range |
|---|---|---|
| White cell count (WBC), eosinophils, hemoglobin, mean corpuscular volume, platelets count, and fasting blood sugar | Normal range | Normal or abnormal |
| Serum creatinine, BUN, uric acid, sodium, potassium, alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyltranspeptidase | Normal range | Normal or abnormal |
| Alkaline phosphatase, albumin, albumin-corrected calcium, thyroid function tests (TSH, free T3, total T3, free T4), serum vitamin B12 and folate levels | Normal range | Normal or abnormal |
| Lactate dehydrogenase | 430 IU/L | 105–333 IU/L |
| C-reactive protein (CRP) | 31.1 mg/dL | 0.0–1.0 mg/dL |
| Antistreptolysin O Titer | 90 IU/mL | 0–200 IU/mL |
| Venereal disease research laboratory (VDRL) test, fluorescent treponema pallidum antibody absorption (FTA-ABS), and ELISA for HIV test | Non-reactive | Nonreactive or reactive |
| Antibodies: Antithyroid peroxidase, herpes virus 1 and 2 IgG and IgM, cytomegalovirus (CMV) IgG and IgM, Epstein–Barr virus (EBV) IgG and IgM, hepatitis BS AG, hepatitis C IgG and IgM, Toxoplasma gondii IgG and IgM, and Echinococcus granulosus IgG | Negative | Positive or negative |
| Anti-double-stranded DNA, antinuclear antibody, antinuclear factor, perinuclear antineutrophil cytoplasmic antibody, cytoplasmic antineutrophil cytoplasmic antibody, and anti-GAD antibody | Negative | Positive or negative |
| Serum measles IGG | 4237.31 IU/L | Positive: ≥275 IU/L |
| Serum measles IGM | 0.09 IU/L | Negative: Less than 0.8 IU/L |
| Urine test | Result | Reference range |
| Physical, macroscopic, and microscopic urine analysis | Normal | Normal or abnormal |
| Urine culture | Negative | Positive or negative |
| An 8-point toxicology screen | Negative for cocaine metabolite, opiates, amphetamine, tetrahydrocannabinol, ethanol, phencyclidine, benzodiazepines, and barbiturates | Positive or negative |
| Cerebrospinal fluid (CSF) investigations: Lumbar puncture performed on day 8th from admission | Result | Reference range |
| Appearance, opening pressure, and glucose | Normal | Normal or abnormal |
| Protein | 58 mg/dL | 5–40 mg/dL |
| Cell count: White blood cells, lymphocytes, polymorphous/pus cells, red blood cells, epithelial cells, yeast cells | Nil | Normal: 0–5 cells/mm3 |
| Gram stain, india ink acid fast, culture, cytology | Negative | Normal: Negative |
| Toxoplasma gondii IgG and IgM antibodies | Negative | Positive or negative |
| Measles IGG in CSF | 4017.91 IU/L | Positive: ≥275 IU/L |
| Measles IGM in CSF | 0.09 IU/L | Negative: Less than 0.8 IU/L |
| VDRL and FTA-ABS | Nonreactive | Nonreactive or reactive |
| India ink test for Cryptococcus neoformans and HTLV 1 and 2, and anti-GAD antibody | Negative | Negative or positive |
| Oligoclonal bands in CSF | Positive 6 oligoclonal bands detected in CSF but none was detected in serum. | Negative or positive |
| Real-time PCR test for Escherichia coli, Hemophilus influenzae, Listeria monocytogenes, Neisseria meningitides, Streptococcus agalactia, Streptococcus pneumonia, Cytomegalovirus DNA, Enterovirus, Herpes simplex virus 1, Herpes simplex virus 2, Human herpes virus 6, human parechovirus, Varicella zoster virus, Cryptococcus neoformans gattii. | Not detected | Detected or not detected |
| Other investigations | Result | Reference range |
| Mantoux test and QuantiFERON test for tuberculosis | Negative | Positive or negative |
| Electrocardiogram, chest X-ray, and echocardiogram | Normal | Normal or abnormal |
| Abdominal and pelvic ultrasound, and KUB ultrasound | Uterine fibroid otherwise normal | Normal or abnormal |
| CT scan of the brain, chest, abdomen, and pelvis with contrast and MRA/MRV scan of the brain | Normal | Normal or abnormal |
| Magnetic resonance imaging (MRI) scan of the brain | Abnormal | Normal or abnormal |
| Scalp electroencephalogram (EEG) | Abnormal | Normal or abnormal |
| Electromyography (EMG) and nerve conduction studies, video-EEG, polysomnography, and jerk-locked back averaging studies | Tests not obtained | Normal or abnormal |
[i] Abbreviations: BUN, Blood Urea Nitrogen; CSF, Cerebrospinal Fluid; CT, Computed Tomography; DNA, Deoxyribonucleic Acid; ELISA, Enzyme-Linked Immune Sorbent Assay; Free T3, Free Triiodothyronine; Free Total T3, Free Total Triiodothyronine; Free T4, Free Thyroxine; HPF, Microscopic High Power Field; MRA, Magnetic Resonance Angiography; PCR, Protein Chain Reaction; TSH, Thyroid-Stimulating Hormone.
Figure 1
Photographs of the MRI scan of the brain of the patient with SSPE. (A) Sagittal magnetic resonance imaging (MRI) T1-weighted image of the brain showing diffuse atrophy. (B) Axial T1-weighted post-IV gadolinium showing mild subcortical enhancement in the right parietal lobe (blue arrows). (C) Axial MRI T2-weighted image of the brain showing asymmetric hyperintensities best noted in the right temporoparietal region (blue arrows). (D) Axial MRI T2-FLAIR-weighted image of the brain showing diffuse hyperintensities in mesial temporal lobes bilaterally (blue arrows). (E) Axial MRI T2-FLAIR-weighted image with hyperintensities best seen in the frontal lobes (blue arrows) bilaterally.
Video 1
Phenomenology: Segment 1. The patient with SSPE at admission: The involuntary motor activity consisted of abnormal, sudden, segmental, brief multifocal, and predominantly distal muscle jerks involving the patient’s upper limbs more on the left than the right side. The phenomenon was observed purely in wakefulness. It was accompanied by dystonia of both legs with the knees flexed at 90°. The patient tried to stop the abnormal movement disorder unsuccessfully using the right hand which was only partially involved. The myoclonic jerks usually commenced sharply in the first hour of awake and remained unchanged throughout wakefulness. There was an observable pattern of one to two sequences of muscle contractions every 2–3 seconds continuously. This phenomenon occurred numerous times every day for 15 days.
Video 1
Segment 2. The patient from Segment 1 at Follow-up: These myoclonic jerks, dystonia, and spasticity responded completely to the treatment with a combination of clonazepam 0.5 mg and valproic acid 200 mg orally twice daily. At 3 months of follow-up and with compliance to treatment, these movements have not returned.