Video 1
Pre- and Post-Treatment Video of the Patient.
Phenomenology
0:00–0:08 – Bilateral slow, writhing finger and hand movements at rest.
0:08–0:16 – Marked increase in amplitude and dystonic posturing with eyes closed.
0:17–0:26 – Post-plasmapheresis: complete resolution of movements.
0:26–0:29 – No re-emergence on eye closure.
Table 1
Baseline laboratory investigations of the patient.
| INVESTIGATIONS | PATIENT’S VALUES | REFERENCE RANGE |
|---|---|---|
| Hemoglobin (g/dl) | 12.9 | 12–15 |
| Total Leukocyte count (× 10^3/µL), | 9.8 | 4–10 |
| Neutrophil %/Leukocyte % | 62/25 | 40–80/20–40 |
| Platelet count (× 10^3/µL) | 273 | 150–410 |
| Prothrombin time(s)/INR | 13.5/1.1 | 11–16/0.8–1.1 |
| ALT/AST (U/L/ U/L) | 27/48 | 14–36/10–49 |
| Creatinine/Urea (mg/dl/mg/dl) | 0.6/37 | 0.52–1.04/15–42 |
| HbA1C(%) | 5.2 | <5.7 |
| Lipid profile/T3/T4/TSH | Normal | |
| Vitamin B12 (pg/ml) | 914 | 197–771 |
| Serum Folate (ng/ml) | 8.7 | 3.1–17.5 |
| Serum Homocysteine (umol/L) | 14.7 | 0–15 |
| ANA/ENA/ANCA (IIF) | Negative | |
| Serum ACE levels | Normal | |
| HIV/HbsAg/anti-HCV | Negative | |
| Serum Anti-Aquaporin 4 IgG (Fixed CBA-IIF) | Strong positive | |
| Serum MOG IgG (Fixed CBA-IIF) | Negative | |
| CSF- cells/uL, Neutrophil %/Lymphocyte % | Nil | 0-5 cells/uL |
| CSF- protein (mg/dl) | 36 | 15–45 |
| CSF-sugar (mg/dl) | 64/110 | 40–70 |
| CSF- gram stain, culture, India Ink, Cryptococcal Antigen, VDRL, Acid fast bacilli staining, Gene Xpert, Malignant cytology | Negative | |
| Serum Paraneoplastic Panel (Immunoblot) Anti Hu, Anti Ri, Anti Yo, Anti CV2, Anti Ma2/Ta, Anti Amphiphysin, Anti GAD 65, Anti Zic 4, Anti titin, Anti Recoverin | Negative | |
| Serum and CSF Autoimmune Panel (Fixed CBA-IIF) NMDA, AMPA 1,2, CASPR2, LGI1, GABA B | Negative |
[i] *CBA-IIF: Cell-Based Assay- Indirect Immunofluorescence, ANA: Anti-Nuclear Antigen, ENA: Extractable Nuclear Antigen, ANCA: Anti-Neutrophilic Cytoplasmic Antibody, CSF: Cerebrospinal Fluid, MOG- Myelin Oligodendrocyte Glycoprotein, NMDA: N- Methyl-D-Aspartate, AMPA: α-amino-3-hydroxy-5-methyl-4-isoxazoleproprinoic acid, CASPR2: Contactin associated protein type 2, LGI1: Leucine Rich Glioma Inactivated Protein-1, GABA-B: Gamma-Aminobutyric Acid-B.
Figure 1
MRI demonstrating isolated medullary involvement. (A) Sagittal T2-FLAIR image showing a focal hyperintense lesion in the dorsal medulla (arrow), with no signal abnormality in the cervicomedullary junction. (B) Axial T2-FLAIR image demonstrating the corresponding medullary hyperintensity (arrow). (C) Sagittal T2-weighted image of the cervico-dorsal spinal cord showing no signal abnormality.