Clinical features during the attack of HMPA
| Patient, current age (years) | Patient 1 (III-1), 45 | Patient 2 (II-3), 72 | Patient 3 (III-2), 42 | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Serial no of HMPA, age (years) | 1st, 33 | 2nd, 35 | 3rd, 36 | 4th, 40 | 5th, 42 | 6th, 42 | 7th, 44 | 1st, 66 | 2nd, 68 | 1st, 41 | 2nd, 41 |
| BT at admission (°C) | 38.5 | 37.8 | 38.2 | 38.5 | 38.1 | 39.1 | 38.1 | 38.9 | 39.0 | 37.0 | 38.5 |
| Side of affected hemisphere | R | L | L | L | R | L | L | R | R | L | L |
| 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, R HP, aphasia, R VFD, VH, Psy | Confusion, L HP, L VFD, VH, Psy | Confusion, L HP, L VFD, VH, Psy | Confusion, R HP, R HSD, aphasia, R VFD | Confusion, aphasia, R VFD |
| Aura duration (days) | 5 | 10 | 4 | 8 | 7 | 7 | 8 | 12 | 11 | 4 | 4 |
| CBF-SPECT (day, tracer) | Decreased in R F, T, P with CCD (2, HMPAO), normal (8, HMPAO) | Increased in L F, T, P, O with CCD (2, 4, HMPAO), normal (23, HMPAO) | Increased in L F, T (2, IMP) | Increased in L F (2, IMP), increased in L P (8, IMP) | Decreased in R F, T (1, IMP) | Decreased in L F, T, P with CCD (1, IMP), normal (13, IMP) | Decreased in L F, T, P, O with CCD (1, IMP), mildly increased in L O, T (9, IMP) | Increased in R T, P, O with CCD (2, IMP), normal (10, IMP) | Increased in R T, P, O (3, IMP), normal (10, IMP) | Increased in L F, T, P, O with CCA (3, IMP) | Decreased in L F, T, P, O with CCD (1, IMP), mildly increased in L T (7, IMP) |
| ASL-MRI (day) | NA | NA | NA | NA | NA | NA | Increased in L F, T, P, O with CCA (3), increased in L T, P, O with CCA (4), no laterality (9) | NA | NA | Increased in L F, T, P, O (2), no laterality (6) | Decreased in L F, T, P, O with CCD (1), increased in L F, T, P, O (2), no laterality (10) |
| Demonstration of MCA branches on MRA (day) | Prominently increased (3) | Prominently increased (3) | Mildly increased (2) | Mildly decreased (1) | Normal (1) | Normal (1) | Normal (1), mildly increased (3, 4, 9) | Mildly increased (2) | Mildly increased (2) | Normal (2, 6) | Mildly decreased (1), mildly increased (2), normal (10) |
| DWI (day) | Normal (3) | Normal (2) | Normal (2) | Normal (1) | Normal (1) | Normal (1) | Normal (1, 3, 4, 9) | Normal (2) | Normal (2) | Normal (2, 6) | Normal (1, 2, 10) |
| EEG (day) | LVA in R hemisphere (4) | LVA in L. hemisphere (3) | LVA in L hemisphere (2) | LVA in L hemisphere (3) | NA | LVA in L hemisphere and delta slowing in R hemisphere (1)*, LVA in L hemisphere (3) | LVA in L. hemisphere with slowing in R hemisphere (2)*, LVA in L hemisphere (9) | LVA in R hemisphere (2) | LVA in R hemisphere (3) | No laterality, small spike in T (2) | LVA in L hemisphere (1) |
| Abortive therapy (day of initiation) | None | IV furosemide 20 mg (2), acetazolamide 750 mg/day (2), PSL 60 mg/day (4) | None | IVMP 1000 mg, 3 days (1), followed by short taper-off | IVMP 1000 mg 3 days (1), followed by short taper-off | PSL 60 mg/day (1), followed by short taper-off | PSL 60 mg/day (1), followed by short taper-off | IVMP 1000 mg, 3 days (2) | PSL 60 mg/day (2), followed by short taper-off | PSL 40 mg/day (2), followed by short taper-off | PSL 50 mg/day (1), followed by short taper-off |
*EEG recorded after IV infusion of propofol to suppress psychiatric symptoms. Both crossed cerebellar hypoperfusion and hypermetabolism are shown as CCA. Day 1 is the first day of the onset of aura symptoms.
AH, auditory hallucination; BT, body temperature; CBF, cerebral blood flow; CCA, crossed cerebellar activation; CCD, crossed cerebellar diaschisis; DWI, diffusion-weighted MRI, F, frontal; HMPA, hemiplegic migraine with prolonged aura; HMPAO, 99mTc-d,l-hexamethyl-propyleneamine oxime; HP, hemiparesis; HSD, hemisensory deficit; IMP, N-isoprpyl-p-123I iodoamphetamine; IVMP, intravenous high-dose methylprednisolone; L, left; LVA, low-voltage activity; MCA, middle cerebral artery; NA, not available; O, occipital; P, parietal; PSL, prednisone; Psy; psychiatric symptoms; R, right; T, temporal; VFD, visual field defect; VH, visual hallucination.