Clinico-radiological features during attacks of hemiplegic migraine with prolonged aura (HMPA)
| Patient, relationship, current age (years), gender | Patient 1, proband (III-1), 42, F | Patient 2, mother (II-3), 69, F | ||||||
| Serial no of HMPA, age (years) | 1st, 33 | 2nd, 35 | 3rd, 36 | 4th, 40 | 5th, 42 | 6th, 42 | 7th, 66 | 8th, 68 |
| BT at admission (°C) | 38.5 | 37.8 | 38.2 | 38.5 | 38.1 | 39.1 | 38.9 | 39.0 |
| Affected hemisphere* | R | L | L | L | R | L | R | R |
| Neurological manifestations | Confusion, L HP, VH, Psy | Confusion, R HP, aphasia, R VFD, VH, Psy | Confusion, R HP, aphasia, R VFD, Psy | Confusion, R HP, aphasia, R VFD, VH, Psy | Confusion, L HP, L VFD, VH, Psy | Delirium, R HP, aphasia, R VFD, VH, AH, Psy | Confusion, L HP, L VFD, VH, Psy | Confusion, L HP, L VFD, VH, Psy |
| Duration of aura (days) | 5 | 10 | 4 | 8 | 7 | 7 | 12 | 11 |
| SPECT during active phase of aura symptoms (day, tracer) | Decreased in R F, T, P with CCD (2, HMPAO) | Increased in L F, T, P, O with CCD (2, 4, HMPAO) | Increased in L F, T with CCD (3, IMP) | Increased in L F with CCD (2, IMP) | Decreased in R F, T without CCD (2, IMP) | Decreased in L F, T, P with CCD (1, IMP) | Increased in R T, P, O with CCD (2, IMP) | Increased in R T, P, O without CCD (3, IMP) |
| Follow-up SPECT during recovery or after resolution of symptoms (day, tracer) | Normal (8, HMPAO) | Normal (23, HMPAO) | NA | Increased in L P without CCD (8, IMP) | NA | Normal (13, IMP) | Normal (10, IMP) | Normal (10, IMP) |
| Demonstration of MCA branches on MRA (day) | Prominently increased (3) | Prominently increased (3) | Mildly increased (3) | Mildly decreased (1) | Normal (2) | Normal (1) | Mildly increased (2) | Mildly increased (2) |
| DWI (day) | Normal (3) | Normal (2, 4) | Normal (3) | Normal (1) | Normal (2) | Normal (1) | Normal (2) | Normal (2) |
| Enhanced FLAIR early image† (day) | NA | Mild cortical oedema (4) | Normal (3) | Normal (1) | Normal (2) | NA | Normal (2) | Normal (2) |
| Enhanced FLAIR late image‡ (day) | NA | Cerebrospinal fluid enhancement in affected cortex (4) | No enhancement (3) | NA | NA | NA | No enhancement (4) | NA |
| H-MRS (day) | NA | NA | NA | NA | Normal (3) | NA | NA | Normal (2) |
| EEG during active phase of aura symptoms (day) | LVS in R hemisphere (4) | LVS in L hemisphere (3) | LVS in L hemisphere (3) | LVS in L hemisphere (3) | NA | LVS in L hemisphere and delta slowing in R hemisphere (1)§ | LVS in R hemisphere (2) | LVS in R hemisphere (3) |
| Abortive therapy during acute stage (day of initiation date of therapy)¶ | None | Intravenous furosemide 20 mg, (2), acetazolamide 750 mg/day (2), PDN 60 mg/day (4) | None | IVHM 1000 mg, 3 days (1), followed by short taper-off | IVHM 1000 mg, 3 days (2), followed by short taper-off | PDN 60 mg/day (1) | IVMP 1000 mg, 3 days (2) | PDN 60 mg/day (2) |
↵* Affected hemisphere is defined as hemisphere contralateral to hemiplegia.
↵† Enhanced fluid-attenuated inversion recovery (FLAIR) early image was a conventional enhanced FLAIR image obtained 5–10 min after intravenous injection of gadolinium.
↵‡ Enhanced FLAIR late image was obtained 2 h after intravenous injection of gadolinium.
↵§ EEG recording under continuous intravenous infusion of propofol (0.42 mg/kg/h) to suppress psychiatric symptoms (delta slowing in the right hemisphere resolved after discontinuation of propofol, but low-voltage slowing persisted in the left hemisphere on day 3).
↵¶ The first imaging studies were obtained before administration of corticosteroids.
AH, auditory hallucination; BT, body temperature; CCD, crossed cerebellar diaschisis; DWI, diffusion-weighted MRI; F, frontal; HMPAO, 99mTc-d,l-hexamethyl-propyleneamine oxime; HP, hemiparesis; IMP, N-isopropyl-p-123I iodoamphetamine; IVMP, intravenous high-dose methylprednisolone; L, left; LVS, low-voltage slowing; MCA, middle cerebral artery; MRS, MR spectroscopy; NA, not available; O, occipital; P, parietal; PDN, prednisone; Psy, psychiatric symptoms; R, right; T, temporal; VFD, visual-field defect; VH, visual hallucination.