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Table 4 Therapeutic effects improvement/symptoms

From: ENT characteristics and therapeutic results in multisystemic disorders of mitochondrial encephalomyopathy

 

TX

CPEO (N %)

MELAS (N %)

KSS (N %)

MIDD (N %)

LHON (N %)

Total (N %)

Otolaryngology

 Hearing loss

HA/CI

1/6(16.7)

1/5(20)

1/4(25)

1/4(25)

0/0(0)

4/19(21.1)

 Tinnitus

HA/CI

0/1(0)

1/2(50)

0/2(0)

1/1(100)

0/0(0)

2/6(33.3)

 Auditory agnosia

RX

0/0(0)

1/1(100)

0/0(0)

0/0(0)

0/0(0)

1/1(100)

 Dysphagia

MILL’S

1/5(20)

0/0(0)

0/1(0)

0/0(0)

0/0(0)

1/6(16.7)

 Facial weakness

N

0/4(0)

0/0(0)

0/0(0)

0/0(0)

0/0(0)

0/(0)

Ophthalmology

 Ptosis

CFS

8/15(53.3)

0/1(0)

2/5(40)

0/0(0)

0/0(0)

10/21(47.6)

 Extraocular muscle paralysis

EOMS

6/12(50)

0/1(0)

1/5(20)

0/0(0)

0/0(0)

7/18(38.9)

 Retinitis pigmentosa

IDBN/GT

0/0(0)

0/1(0)

1/4(25)

0/0(0)

1/3(33.3)

2/8(25)

 Optic atrophy

IDBN/GT

0/0(0)

0/2(0)

0/3(0)

0/0(0)

2/3(66.7)

2/8(25)

 Cataract

PHACO + IOL

3/3(100)

1/3(33.3)

0/0(0)

0/0(0)

0/0(0)

4/6(66.7)

 Eye pain

RX

0/0(0)

3/3(100)

0/0(0)

0/0(0)

0/0(0)

3/3(100)

 Hemianopsia

N

0/0(0)

0/3(0)

0/0(0)

0/0(0)

0/0(0)

0/3(0)

 Glaucoma

RX

1/1(100)

1/2(50)

0/0(0)

0/0(0)

0/0(0)

2/3(66.7)

 Diplopia

RX

1/2(50)

0/0(0)

0/0(0)

0/0(0)

0/0(0)

1/2(50)

Neurology

 Exercise intolerance

RX

1/10(10)

1/2(50)

1/4(25)

0/0(0)

0/0(0)

3/16(18.8)

 Stroke-like episode

RX

0/0(0)

5/5(100)

0/0(0)

0/0(0)

0/0(0)

5/5(100)

 Mental retardation

N

0/0(0)

0/1(0)

0/2(0)

0/2(0)

0/0(0)

0/5(0)

 Headache

RX

0/0(0)

3/3(100)

0/0(0)

0/0(0)

0/0(0)

3/3(100)

 Palpitation

RX

0/3(0)

0/3(0)

1/3(33.3)

0/0(0)

0/0(0)

1/9(11.1)

 Muscular atrophy

N

0/4(0)

0/1(0)

0/2(0)

0/0(0)

0/0(0)

0/7(0)

 Myoclonic seizures

RX

0/0(0)

1/4(25)

0/0(0)

0/0(0)

0/0(0)

1/4(25)

 Abnormal mental behavior

N

0/0(0)

0/2(0)

0/2(0)

0/0(0)

0/0(0)

0/4(0)

 Dizziness

RX

0/0(0)

0/0(0)

0/0(0)

2/3(66.7)

0/0(0)

2/3(66.7)

 Limb numbness

RX

0/0(0)

0/0(0)

0/0(0)

2/3(66.7)

0/0(0)

2/3(66.7)

 Aphasia

RX

0/0(0)

1/2(50)

0/0(0)

0/0(0)

0/0(0)

1/2(50)

 Insomnia

RX

0/0(0)

0/0(0)

1/1(100)

0/0(0)

0/0(0)

1/1(100)

None-Neuromuscular system

 Diabetes

RX

0/0(0)

0/0(0)

0/0(0)

4/4(100)

0/0(0)

4/4(100)

 Acute fever

RX

0/0(0)

3/3(100)

1/1(100)

0/0(0)

0/0(0)

4/4(100)

 Hypermicrosoma

N

0/2(0)

0/1(0)

0/1(0)

0/0(0)

0/0(0)

0/4(0)

 Low BMI

N

0/2(0)

0/1(0)

0/1(0)

0/0(0)

0/0(0)

0/4(0)

 Vomit

RX

0/0(0)

2/2(100)

1/1(100)

0/0(0)

0/0(0)

3/3(100)

 Diarrhea or constipation

RX

1/1(100)

1/1(100)

0/0(0)

0/0(0)

0/0(0)

2/2(100)

 Depression

RX

2/2(100)

0/0(0)

0/0(0)

0/0(0)

0/0(0)

2/2(100)

 Kidney damage

RX

0/0(0)

0/0(0)

0/0(0)

1/1(100)

0/0(0)

1/1(100)

 Hypogonadism

N

0/1(0)

0/0(0)

0/0(0)

0/0(0)

0/0(0)

0/1(0)

  1. TX treatment, CPEO chronic progressive external ophthalmoplegia, MELAS mitochondrialencephalomyopathy lactic acidosis stroke-like episodes, KSS Kearns-Sayre syndrome, MIDD maternally inherited diabetes and deafness, LHON Leber hereditary optic neuropathy, HA hearing aid, CI cochlear implantation, RX receptor X(coenzyme Q10/ L-carnitine/arginine/lipoic acid), MILL’S microlaryngoscopic laser surgery, N none, correctin of ptosis andcanthoplasty, CFS conjoint fascial sheath, EOMS extraocular muscle surgery, IDBN Idebenone, GT gene therapy, PHACO phacoemulsification, IOL intraocular lens