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Ann Child Neurol > Volume 29(3); 2021 > Article |
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Author contribution
Conceptualization: JL and JL. Data curation: JYS and JL. Formal analysis: JYS. Methodology: JIL, HJS, JL, and JL. Project administration: JL and JL. Visualization: JYS. Writing-original draft: JYS. Writing-review & editing: JL and JL.
Case | Sex | Age at diagnosis | FU (yr) |
Presenting symptom (onset age) |
Response to ASM | HH size (long axis, cm) | HH typea | Neuropsychological disorder | |
---|---|---|---|---|---|---|---|---|---|
Seizure | PP | ||||||||
1 | F | 8 mo | 18.6 | GS (8 mo) | PP (8 mo) | None | 2 | M | None |
FSIA,GTC (8 mo) | |||||||||
2 | F | 6 yr | 11.3 | FSIA (6 yr) | PP (6 yr) | None | 2.7 | M | Mild ID: FSIQ 75 |
3 | M | 3 yr | 11.8 | GS (3 yr) | PP (6.5 yr) | None | 1.8 | M | Mild ID: FSIQ 81 |
FSIA,GTC (6 yr) | ADHD | ||||||||
4 | M | 3.5 yr | 5 | GS (1 mo) | - | Partial response | 1.8 | M | None |
5 | F | 2.5 yr | 8 | - | PP (2.5 yr) | NA | 2 | P | None |
6 | M | 10.5 yr | 6.7 | - | PP (10.5 yr) | NA | 1 | P | None |
7 | M | 5 yr | 9.9 | GS (3.8 yr) | PP (9 yr) | None | 1 | M | Moderate ID: FSIQ 46 |
FSIA, GT (8 yr) | |||||||||
8 | M | 1 mo | 10.3 | - | - | NA | 2 | P | None |
9 | F | 8 yr | 12.2 | GS (1 mo) | - | None | 1.2 | I | Mild ID: FSIQ 75 |
FSIA (13 yr) | Paranoid PD | ||||||||
10 | F | 2.7 yr | 8.2 | - | PP (2.7 yr) | NA | 1.6 | P | None |
11 | M | 1 yr | 11.6 | GS (1 yr) | - | None | 1.1 | I | None |
12 | F | 4 yr | 2.5 | GS (4 yr) | PP (7.5 yr) | Partial response | 0.9 | M | None |
13 | F | 8 mo | 0.4 | GS (3 mo) | - | None | 2 | M | None |
14 | M | 26 mo | 1 | GS (26 mo) | PP (3.3 yr) | None | 1.2 | M | None |
15 | F | 3.4 yr | 1.5 | - | PP (4 yr) | NA | 1.3 | P | None |
16 | M | 10 yr | 0.3 | GS (7 yr) | - | Partial response | 0.9 | M | None |
17 | M | 2 yr | 1.9 | GS (8 mo) | - | Partial response | 0.8 | M | None |
18 | M | 5 yr | 0.3 | - | PP (5 yr) | NA | 0.8 | I | None |
FU, follow-up; PP, precocious puberty; ASM, anti-seizure medication; HH, hypothalamic hamartoma; GS, gelastic seizure; FSIA, focal seizure with impaired awareness; GTC, generalized tonic clonic seizure; M, mixed hypothalamic; ID, intellectual disability; P, parahypothalamic; FSIQ, full scale intelligence quotient; ADHD, attention deficit hyperactivity disorder; NA, not available; I, intrahypothalamic; PD, personality disorder.
a HH types according to Kameyama et al. [16].
Case |
HH on brain MRI |
Operation (age, yr) | FU after last operation (yr) | Radiation dose (Gy) |
Size change of HH after operation (maximum diameter, mm) |
Seizure outcomes after operation | ||||
---|---|---|---|---|---|---|---|---|---|---|
Sizea (mm) | Typeb | Type of seizure: reduction of frequency |
No. of ASMs |
Engel’s/ILAE classificationc | ||||||
Pre | Post | |||||||||
1 | 20 | M | STR (1.5) | - | - | -d | GS: <10% | 1 | 1 | ID/Class 4 |
GKS (13.2) | 6 | 15 | No change | FSIA, GTC: seizure-free | 1 | 1 | ||||
2 | 27 | M | GKS (7) | 11 | 12 | No change | FSIA: seizure-free | 1 | None | IA/Class 1 |
3 | 18 | M | GKS (6.5) | - | 17 | Minimal decrease (17→after 4 years: 10) | GS: >50% | 1 | None | ID/Class 4 |
RFA (15.1) | - | - | >50% decrease (7) | FSIA, GTC: seizure-free | 2 | 1 | ||||
GKS (17.1) | 1 | 18 | No change | 1 | 2 | |||||
4 | 18 | M | GKS (4.8) | 4 | 17 | Minimal decrease (16) | GS: seizure-free | 3 | None | IA/Class 1 |
7 | 10 | M | GKS (6) | - | 17 | No change | GS: >50% | 4 | 4 | IVC/Class 6 |
ED (7.4) | 8 | >50% decrease (10) | FSIA, GT: newly appeared | 4 | 1→5 | |||||
9 | 12 | I | GKS (15.5) | 3 | 17 | Minimal decrease (11.5→after 4 years: 8) | GS: <10% | 3 | 2 | IA/Class 1 |
FSIA: seizure-free | ||||||||||
11 | 11 | I | GKS (6.9) | - | 18 | Minimal decrease (9) | GTC: seizure-free | 2 | 3 | IA/Class 1 |
GKS (9.7) | 2 | 17 | Minimal decrease (7) | GS: seizure-free | 3 | 2 | ||||
12 | 9 | M | GKS (5.1) | - | 18 | No change | GS: <50%→free for 4 months | 2 | 1 | IB/Class 4 |
SRT (7.1) | 0.3 | - | - | 1 | 1 | |||||
17 | 9 | M | SRT (3.2) | 2 | - | >50% decrease (not visible) | GS: recur after 6 months | 2 | None | IB/Class 4 |
FSIA: seizure-free |
HH, hypothalamic hamartoma; MRI, magnetic resonance imaging; FU, follow-up; ASM, anti-seizure medication; ILAE, International League Against Epilepsy; M, mixed hypothalamic; STR, subtotal resection; GKS, gamma-knife radiosurgery; GS, gelastic seizure; FSIA, focal seizure with impaired awareness; GTC, generalized tonic clonic seizure; RFA, radiofrequency ablation; ED, endoscopic disconnection; GT, generalized tonic seizure; I, intrahypothalamic; SRT, stereotactic radiofrequency thermocoagulation.
b HH types according to Kameyama et al. [16];
Study | Year | Treatment | Follow-up (median) | No. of patients | Age at operation (median) | Size of Hha (median) | Seizure outcomesb | Side effects (no. or % of cases) |
---|---|---|---|---|---|---|---|---|
Curry et al. [8] (Texas) | 2011-2018 | LiTT (MRI-guided) | 12 mo (12) | 71 | 5 mo-20 yr | (4-30 mm) | Free from GS: 93% | DI (1) |
Free from seizure and medication: 12% | Severe deficit of short-term memory (1-history of right temporal lobectomy) | |||||||
Episodic hyponatremia (3) | ||||||||
Gadgil et al. [31] (Texas) | 2011-2017 | LiTT (MRI-guided) | 1.2 yr (0.6-6.3) | 58 | 5.5 yr (0.4-20.9) | 0.52 mL (0.06-14.49) | Engel I+II: 81.1% (69.0%+12.1%) | Disorders of sodium metabolism (6.9%) |
Memory disturbance (8.6%) | ||||||||
Unfavorable catheter positioning (1.7%) | ||||||||
Ferrand et al. [32] (Rothschild) | 1998-2017 | ED | 37 mo (13-77) | 112 | 7.6 yr (48-133 mo) | NA | Engel I+II: 77.6% (57.1 %+20.5 %) | DI: transient (2), permanent (1) |
CN III palsy: transient (9), permanent (3) | ||||||||
Memory deficit (4), motor deficit (5), | ||||||||
SUDEP (1), weight gain (5) | ||||||||
Shirozu et al. [12] (Niigata) | 1997-2013 | RFA | 3 yr (1-17) | 100 | 10.0 yr (1-50) | 15 mm (5-80) | Free from GS: 86.0% | Delayed precocious puberty (9.0%) |
Free from other types of seizures: 78.9% | Pituitary dysfunction (2.0%) | |||||||
Weight gain (7.0%) | ||||||||
Regis et al. [33] (Marseille) | 1999-2007 | GKS | 71 mo (36-153) | 48 | 16.5 yr (3-50) | 9 mm (4-30) | Engel I+II: 65% (47.5%+17.5%) | No permanent neurologic side effects |
Transient poikilothermia (6.2%) | ||||||||
Transient seizure increase (16.6%) | ||||||||
Abla et al. [34] (Barrow) | 2003-2010 | GKS | Mean: 43 mo (18-81) | 10 | Mean: 15.1 yr (5.7-29.3) | Mean: 0.2 mL (0.14-0.28) | Free from seizures: 60% | Short-term memory loss (3) |
50%-90% reduction: 10% | Poikilothermia (1) | |||||||
50% reduction: 20% | Increased depression (1) | |||||||
Weight gain/increased appetite (2) | ||||||||
Anxiety (1) | ||||||||
Mathieu et al. [35] (Sherbrooke) | Report from 2010 | GKS | 36 mo (6-56) | 9 | 23 yr (12-57) | 0.6 mL (0.3-1.0) | Engel I: 4 (44.4%) | No side effects after the procedure |
Engel II: 1 (11.1%) |
HH, hypothalamic hamartoma; LiTT, laser interstitial thermal therapy; MRI, magnetic resonance imaging; GS, gelastic seizure; DI, diabetes insipidus; ED, endoscopic disconnection; NA, not available; CN, cranial nerve; SUDEP, sudden unexpected death in epilepsy; RFA, radiofrequency ablation; GKS, gamma knife radiosurgery.
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