| No. | Patient No. | Authors, year | Age/Sex | Height (cm)/Weight (kg)/BMI | Presenting symptoms | Rapid obesity | Hypothalamic dysfunction | Hypoventilation | Autonomic dysregulation | Behavioral changes | Neurologic findings | Neural crest tumor | Other findings | Na (mmol/L) | Prolactin (ng/mL) | fT4 (ng/dL) | Treatment | Outcome |
| 1 | 1 | Park, 2010 [7] | 13/M | 161/70.6/28 | Pain on both thighs, gait disturbance, general weakness, cold body sensation | No | Yes | Yes | Yes | No | Seizure | Ganglio- neuroma | Rhabdo- myolysis | 198 | 35.8 > 13. | 0.5 > 0.8 | Hydration, IVIG | Alive |
| 2 | 2 | Thaker, 2015 [12] | 2/M | 163 (11 yr)/166.3 (11 yr)/62 | - | No | No | No | Yes | Yes | No | No | No | Normal | - | 1.04 | - | Alive |
| 3 | 3 | Gordon, 2015 [13] | 4/F | -/-/- | - | No | DI | Sleep apnea | Yes | No | No | Ganglio- neuroblast oma | No | - | Hyper- prolactinemia | Hypo - thyroidism | Caffeine | Alive |
| 4 | 4 | Tellingen, 2015 [14] | 4/F | -/-/- | Rapid weight gain, growth retardation | Yes | DI | Yes | No | No | No | Ganglio- neuroma | No | - | - | Hypo - thyroidism | - | Alive |
| 5 | 5 | Grudnikoff, 2013 [15] | 4/F | -/-/- | Weight gain, growth retardation, irritability, aggression | Yes | Yes | Sleep apnea | No | Yes | No | Ganglio- neuroma | No | - | - | - | - | Alive |
| 6 | 6 | Patwari, 2011 [16] | 8/F | 150/45>80/36 | - | Yes | Precocious puberty | Sleep apnea | Pupil dilatation | Yes | No | Ganglio- neuroblast oma | Pneumonia, scoliosis | 158 | 56 | - | Artificial ventilation | Alive |
| 7 | 7 | Sartori, 2012 [17] | 4/M | -/-/- | - | Yes | Polyuria, polydipsia | Sleep apnea | Yes | Yes | No | No | No | Normal | Hyper- prolactinemia | - | IVIG, artificial ventilation | Alive |
| 7 | 8 | Sartori, 2012 [17] | 5/F | -/-/- | - | Yes | Yes | Yes | No | Yes | No | No | No | - | - | Central hypo- thyroidism | - | Alive |
| 8 | 9 | Dhondt, 2013 [18] | 3/F | -/-/- | Stagnation of neurodevelopment, aggression, hyperphagia | Yes | Precocious puberty | Yes | Yes | Yes | Yes | No | No | - | - | - | Artificial ventilation | Alive |
| 9 | 10 | Bougnères, 2008 [19] | 4/- | -/-22 | - | Yes | Yes | Yes | Yes | Mental retardation, psychosis | No | Ganglio- neuroma | No | 156 | 19 | 8.5 | - | Alive |
| 9 | 11 | Bougnères, 2008 [19] | 3/- | -/-/40 | - | Yes | Yes | Sleep apnea | Yes | Mental retardation | No | Ganglio- neuroma | No | 161 | 39 | 9.8 | - | Alive |
| 9 | 12 | Bougnères, 2008 [19] | 3/- | -/-/29 | - | Yes | Yes | Yes | Yes | No | No | Ganglio- neuroma | No | 150 | 14 | 17.1 | - | Alive |
| 9 | 13 | Bougnères, 2008 [19] | 3/- | -/-/35 | - | Yes | Yes | Sleep apnea | Yes | No | No | Ganglio- neuroma | No | 151 | 22 | 16 | - | Alive |
| 9 | 14 | Bougnères, 2008 [19] | 2/- | -/-/24 | - | No | Yes | Yes | Yes | No | No | Ganglio- neuroma | No | 145 | 31 | 16 | - | Alive |
| 9 | 15 | Bougnères, 2008 [19] | 2/- | -/-/44 | - | No | Yes | Yes | Yes | No | No | Ganglio- neuroma | No | 149 | 34 | 12.4 | - | Alive |
| 10 | 16 | Paz-Priel, 2011 [20] | 5/F | -/-/17 > 25 | - | Yes | Yes | No | Left exotropia | Aggressive behavior | No | Ganglio- neuroblast oma | No | - | 76.5 | Normal | Cyclophosphamide, IVIG, prednisone, rituximab | Alive |
| 11 | 17 | Chandrakantan, 2012 [21] | 5/F | 108/29/25 | - | Yes | DI | Yes | Pupil dilatation, pupil response decrease | No | No | Ganglio- neuroblast oma | No | Hyper Na | Hyper- prolactinemia | - | Artificial ventilation, tracheostomy | Alive |
| 11 | 18 | Chandrakantan, 2012 [21] | 9/F | 137/54/29 | - | Yes | DI | Yes | Chronic constipation, neurogenic bladder | No | Developmental delay | No | No | Hyper Na | - | - | Noninvasive mask | Alive |
| 12 | 19 | Kocaay, 2014 [22] | 13/F | 145/69/32 (10 yrs) | Respiratory distress, cyanosis | Yes | Hypogonadism, secondary amenorrhea, precocious puberty | Yes | No | Social withdrawal | Drowsiness | No | Megaloblastic anemia, acanthosis nigricans, Raynaud phenomenon | 151 | 1.044 (10 yrs) | 0.88 | - | Alive |
| 13 | 20 | Sumanasena, 2012 [23] | 10/F | -/35/- | Progressive respiratory difficulty, edema | Yes | No | Yes | No | Hallucination | Drowsiness | Ganglio- neuroma | No | 167 | Normal | - | - | Alive |
| 14 | 21 | Abaci, 2013 [3] | 3/M | 92 > 95.8 (9 mo)/20 > 25.7 (9 mo)/24>28 | Cyanosis, recent onset dyspnea | Yes | Polyuria, polydipsia | Yes | No | Yes | No | Ganglione Uroblasto ma | No | 143 | 44.7 | 0.75 | Cyclophosphamide, IVIG, dexamethasone, rituximab | Alive |
| 15 | 22 | Atapattu, 2015 [24] | 4/F | -/-/- | Excessive weight gain, increase food seeking, daytime somnolence | Yes | No | Yes | No | Yes | No | Ganglione uroma | Celiac disease | - | - | - | Hypertension medication | Alive |
| 16 | 23 | Uçar, 2013 [8] | 6/F | -/-/- | Blurring of consciousness, recurrent fever | No | GH deficiency | Yes | No | No | Yes | Hamartoma tous mass with neural elements | No | 152 | 89 | - | Desmopressin acetate, ventilatory support | Alive |
| 17 | 24 | Sethi, 2014 [25] | 5/F | 117/25 > 37/14>28 | Behavior outbursts, poor school performance, hyperphagia, fever, abdominal pain with rectal prolapse | Yes | Yes | No | Bilateral tonic pupils | Yes | No | Ganglione uroblasto ma | Metabolic alkalosis | HypoNa | - | Normal | Endotracheal intubation, risperidone, benzodiazepines, antipsychotic medications | Multiorgan failure, death |
| 18 | 25 | Gallizia, 2012 [26] | 3/F | -/-/- | Rapid weight gain, polyuria, sleep apnea | Yes | Yes | Sleep apnea | No | No | No | No | No | - | Hyper- prolactinemia | Normal | - | Alive |
| 18 | 26 | Gallizia, 2012 [26] | 3/F | -/-/- | Rapid weight gain, fatigue, polydipsia, syncope episodes, strabismus, behavioral problems | Yes | Yes | No | Strabismus | Yes | Yes | No | No | - | Hyper- prolactinemia | Normal | Mechanical ventilation | Alive |
| 19 | 27 | Baronio, 2013 [27] | 1/M | -/-/- | Severe obesity, hyperreninemic hypertension | Yes | No | No | No | No | No | Ganglione uroblasto ma | No | - | - | 0.62 | Mechanical ventilation, brain hypothermia, steroid pulse | Alive |
| 19 | 28 | Baronio, 2013 [27] | 2/F | -/-/- | Severe obesity, hyperreninemic hypertension | Yes | GH deficiency | No | No | No | No | Ganglione uroblasto ma | No | - | Hyper- prolactinemia | 0.17 | Mechanical ventilation, brain hypothermia, steroid pulse | Alive |
| 20 | 29 | Chow, 2014 [28] | 15/M | 174/87/29 | Fever, headache, vomiting, weight gain | Yes | No | No | No | Irritability, lethargy, somnolence | No | No | No | 150>123 | - | - | Mechanical ventilation, IVIG, methylprednisolone | Alive |
| 21 | 30 | Kot, 2012 [29] | 9/M | short stature/-/- | Weight gain, short stature, hyperphagia, hypodipsia, thermal dysregulation, excessive perspiration, cold extremity, livedo reticularis, sleep apnea | Yes | Hypodipsia, GH deficiency | Sleep apnea | Yes | No | No | No | No | 161 | Hyper-prolactinemia | Normal | GH replacement | Alive |
| 22 | 31 | Cemeroglu, 2015 [30] | 5/- | -/-/- | Short stature, obesity | Yes | DI, GH deficiency | Sleep apnea | Yes | Flat affect | No | No | Scoliosis | 157>153 | - | 0.9>0.5 | GH replacement, levothyroxine, desmopressin, tonsillectomy, adenoidectomy, CPAP | Alive |
| 23 | 32 | Chew, 2011 [31] | 11/M | -/35/26 | Fever, drowsiness, shallow breathing | No | DI, hypogonadism | Sleep apnea | Thermal dysregulation, excessive sweating, right divergent squint | 0 | Seizure, developmental delay | No | Respiratory acidosis | 192 | Hyper-prolactinemia | Normal | Anti-epileptics | Alive |
| 24 | 33 | Petty, 2014 [32] | -/M | -/-/- | Weight gain, enuresis, sleep apnea, fever | Yes | No | Yes | Transient visual loss | Hallucination | No | No | Thrombocy topenia | - | - | - | IVIG, CPM, rituximab | Alive |
| 25 | 34 | Maksoud, 2015 [33] | 6/F | 119/38/27 | Abdominal mass, rapid onset obesity | Yes | Premature thelarche, GH deficiency | Sleep apnea | Urinary incontinence | No | No | Ganglione uroma | Hepatitis C | 156 | - | 0.79 | - | Alive |
| 26 | 35 | Sanklecha, 2016 [34] | 2/F | -/-/- | Gait disturbance, head jerky movement, nystagmus | Yes | DI, polyuria, polydipsia | Yes | No | Aggressiveness | Seizure | Ganglione uroblasto ma | No | 189>115 | - | - | Chemotherapy, mechanical ventilation, tracheostomy, nasal BIPAP, rituximab, CPM | Cardiac arrest, sudden demise |
| 27 | 36 | Erensoy, 2016 [35] | 8/F | -/-/- | Overweight, recession, fatigue, decreased school success | Yes | No | No | No | Poor school performance, MDD, ADHD | No | No | No | - | - | - | Fluoxetine, methylphenidate | Alive |
| 28 | 37 | Al-Harbi, 2016 [36] | 8/F | 126/45/28 | Progressive fatigue, skin bluish discoloration, fever | Yes | Breast enlargement | Shortness of breath, sleep apnea | Cold intolerance, excessive sweating, altered pain sense | Slow mental function, poor school performance, sleepiness | No | No | No | 186 | Hyper-prolactinemia | Normal | BIPAP | Alive |
| 29 | 38 | Aljabban, 2016 [37] | 4/F | 110/25/- | Rapid weight gain, excessive eating | Yes | polyuria, polydipsia | Sleep apnea | Cold extremity, GI dysmotility | Mood alteration, anxiety, aggressiveness, recurrent fatigue, social withdrawal, sleepiness | Seizure | Ganglio- neuroma | No | Normal > 162 | - | Normal | Antipsychotics, mechanical ventilation, tracheostomy | Cardiac arrest, death |
| 30 | 39 | Bagheri, 2017 [4] | 5/F | 120/40/- | Cough, cyanosis | Yes | Central hypothyroidism | Sleep apnea | Cold extremities, hyperhidrosis, constipation | Mood change | Seizure | Ganglio- neuroblast oma | Facial plethora, buffalo neck | 125 | Hyper-prolactinemia | 0.8 | Mechanical ventilation, tracheostomy | Alive |
| 31 | 40 | Galewicz-zielinska, 2012 [38] | 9/- | -/-/- | - | No | No | Mixed sleep apnea | No | Yes | No | No | Tonsillar hypertrophy | - | - | - | BIPAP | Alive |
| 32 | 41 | Jacobson, 2016 [39] | 2/F | -/-/40 | Hyperphagia, weight gain (16.8 > 35.5 kg) | Yes | Partial DI | Sleep apnea | Reduced pain perception, strabismus | Social withdrawal, autism | No | Ganglio- neuroblast oma | Papular rash | - | - | - | Rituximab, cyclophosphamide | Alive |
| 32 | 42 | Jacobson, 2016 [39] | 3/M | -/-/34 | Hyperphagia, weight gain (18 kg for 3 mo) | Yes | Yes | Sleep apnea | excessive sweating, thermal dysregulation, enuresis, altered pain sense, strabismus | Social withdrawal | No | No | No | - | Hyper-prolactinemia | - | Rituximab, CPM | Alive |
| 33 | 43 | Lucas-Herald, 2012 [40] | 1/F | -/32 (3 yr)/22 | Hyperphagia, food stealing | Yes | Hyper-prolactinemia, GH deficiency, water imbalance | Sleep apnea | Altered pain perception | No | No | No | Renal failure | 184 | - | - | BIPAP | Alive |
| 33 | 44 | Lucas-Herald, 2012 [40] | 2/M | -/33/29 | Obesity | Yes | Hyper-prolactinemia, failed GH | Mixed sleep apnea | No | No | No | No | No | - | - | - | BIPAP | Alive |
| 34 | 45 | Ibanez-Mico, 2017 [41] | 2/F | -/-/- | Obesity, increased appetite | Yes | Hyper-prolactinemia, Central hypothyroidism
| Sleep apnea | Yes | Aggression, hyperactivity, impulsivity | Yes | No | Altered pain sense, GI dysmotility | 175 | 166 | 1.05 | IVIG, steroids, cyclophosphamide Tracheostomy | Sudden death |
| 35 | 46 | Isasa, 2018 [42] | 10/M | 136/66.5/34.92 | Seizures (hyponatremia) | Yes | Hyper-prolactinemia Central hypothyroidism | Central hypoventilation Thermal dysregulation | Polydipsia | Aggressiveness | Yes | No | No | Hyper-/hypo- | - | - | - | Alive |
| 36 | 47 | Siraz, 2018 [43] | 7/F | 130/61/36.0 | obesity | Yes | Central hypothyroidism DI, MDD, Central precocious puberty GH deficiency Hyper-prolactinemia Secondary adrenal insufficiency | No | Excessive sweating hypothermia | No | No | No | Pulmonary hyper-tension IQ 65 | 156 | 33 | 0.7 | - | Alive |
| 36 | 48 | Siraz, 2018 [43] | 5/F | 101/31/30.4 | Obesity, seizure | Yes | Central hypothyroidism Hyper-prolactinemia | Central hypoventilation | Yes | Aggressiveness | Yes | No | Central cyanosis IQ of 3 years of age | 164 | 56 | 0.8 | Tracheostomy | Alive |
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