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Table 1 The multiple organ manifestations of 32 MEM patients

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

 

CPEO N (%)

MELAS N (%)

KSS N (%)

MIDD N (%)

LHN N (%)

Total: N (%)

Onset manifestation

 Otolaryngology

  Hearing loss

6(40)

5(100)

4(80)

4(100)

0(0)

19(59.4)

  Tinnitus

1(6.7)

2(40)

2(40)

1(25)

0(0)

6(18.8)

  Auditory agnosia

0(0)

1(20)

0(0)

0(0)

0(0)

1(3.1)

 Ophthalmology

  Ptosis

15(100)

1(20)

5(100)

0(0)

0(0)

21(65.6)

  Extraocular muscle paralysis

12(80)

1(20)

5(100)

0(0)

0(0)

18(56.2)

  Retinitis pigmentosa

0(0)

1(20)

4(80)

0(0)

3(100)

8(25)

  Optic atrophy

0(0)

2(40)

3(60)

0(0)

3(100)

8(25)

  Cataract

3(20)

3(60)

0(0)

0(0)

0(0)

6(18.8)

  Eye pain

0(0)

3(60)

0(0)

0(0)

0(0)

3(9.4)

 Neurology

  Exercise intolerance

12(80)

2(40)

4(80)

0(0)

0(0)

18(56.2)

  Stroke-like episode

0(0)

5(100)

0(0)

0(0)

0(0)

5(15.6)

  Mental retardation

0(0)

1(20)

2(40)

2(66.7)

0(0)

5(15.6)

  Headache

0(0)

3(60)

0(0)

0(0)

0(0)

3(9.4)

 None-Neuromuscular system

  Diabetes

0(0)

0(0)

0(0)

3(100)

0(0)

3(9.4)

Accompanied manifestation

 Otolaryngology

  Dysphagia

5(33.3)

0(0)

1(20)

0(0)

0(0)

6(18.8)

  Facial weakness

4(26.7)

0(0)

0(0)

0(0)

0(0)

4(12.5)

 Ophthalmology

  Hemianopsia

0(0)

3(60)

0(0)

0(0)

0(0)

3(9.4)

  Glaucoma

1(6.7)

2(40)

0(0)

0(0)

0(0)

3(9.4)

  Diplopia

2(13.3)

0(0)

0(0)

0(0)

0(0)

2(6.3)

 Neurology

  Palpitation

3(20)

3(60)

3(60)

0(0)

0(0)

9(28.1)

  Muscular atrophy

4(26.7)

1(20)

2(40)

0(0)

0(0)

7(21.9)

  Myoclonic seizures

0(0)

4(80)

0(0)

0(0)

0(0)

4(12.5)

  Abnormal mental behavior

0(0)

2(40)

2(40)

0(0)

0(0)

4(12.5)

  Dizziness

0(0)

0(0)

0(0)

3(100)

0(0)

3(9.4)

  Limb numbness

0(0)

0(0)

0(0)

3(100)

0(0)

3(9.4)

  Aphasia

0(0)

2(40)

0(0)

0(0)

0(0)

2(6.3)

  Insomnia

0(0)

0(0)

1(20)

0(0)

0(0)

1(3.1)

 None-Neuromuscular system

  Acute fever

0(0)

3(60)

1(20)

0(0)

0(0)

4(12.5)

  Hypermicrosoma

2(13.3)

1(20)

1(20)

0(0)

0(0)

4(12.5)

  Low BMI

2(13.3)

1(20)

1(20)

0(0)

0(0)

4(12.5)

  Vomit

0(0)

2(40)

1(20)

0(0)

0(0)

3(9.4)

  Diarrhea or constipation

1(6.7)

1(20)

0(0)

0(0)

0(0)

2(6.3)

  Depression

2(13.3)

0(0)

0(0)

0(0)

0(0)

2(6.3)

  Kidney damage

0(0)

0(0)

0(0)

1(33.3)

0(0)

1(3.1)

  Hypogonadism

1(6.7)

0(0)

0(0)

0(0)

0(0)

1(3.1)

  1. CPEO chronic progressive external ophthalmoplegia, MELAS mitochondrialencephalomyopathy lactic acidosis and stroke-like episodes, KSS Kearns-Sayresyndrome, MIDD maternally inherited diabetes and deafness, LHON Leber hereditary optic neuropathy