U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Charcot-Marie-Tooth disease type 1C(CMT1C; HMSN1C)

MedGen UID:
75728
Concept ID:
C0270913
Disease or Syndrome
Synonyms: Charcot-Marie-Tooth disease, demyelinating, Type 1C; Charcot-Marie-Tooth disease, type IC; CHARCOT-MARIE-TOOTH NEUROPATHY, TYPE 1C; CMT 1C; CMT, SLOW NERVE CONDUCTION TYPE C; HMSN IC; NEUROPATHY, HEREDITARY MOTOR AND SENSORY, TYPE IC
SNOMED CT: Charcot-Marie-Tooth disease, type IC (4183003)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): LITAF (16p13.13)
 
Monarch Initiative: MONDO:0010995
OMIM®: 601098
Orphanet: ORPHA101083

Definition

For a phenotypic description and a discussion of genetic heterogeneity of autosomal dominant Charcot-Marie-Tooth disease type 1, see CMT1B (118200). [from OMIM]

Clinical features

From HPO
Pes cavus
MedGen UID:
675590
Concept ID:
C0728829
Congenital Abnormality
An increase in height of the medial longitudinal arch of the foot that does not flatten on weight bearing (i.e., a distinctly hollow form of the sole of the foot when it is bearing weight).
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Hypoesthesia
MedGen UID:
6974
Concept ID:
C0020580
Finding
Decreased ability to perceive touch.
Paresthesia
MedGen UID:
14619
Concept ID:
C0030554
Disease or Syndrome
Abnormal sensations such as tingling, pricking, or numbness of the skin with no apparent physical cause.
Polyneuropathy
MedGen UID:
57502
Concept ID:
C0152025
Disease or Syndrome
A generalized disorder of peripheral nerves.
Positive Romberg sign
MedGen UID:
66017
Concept ID:
C0240914
Finding
The patient stands with the feet placed together and balance and is asked to close his or her eyes. A loss of balance upon eye closure is a positive Romberg sign and is interpreted as indicating a deficit in proprioception.
Hyporeflexia
MedGen UID:
195967
Concept ID:
C0700078
Finding
Reduction of neurologic reflexes such as the knee-jerk reaction.
Gait ataxia
MedGen UID:
155642
Concept ID:
C0751837
Sign or Symptom
A type of ataxia characterized by the impairment of the ability to coordinate the movements required for normal walking. Gait ataxia is characteirzed by a wide-based staggering gait with a tendency to fall.
Sensorimotor neuropathy
MedGen UID:
207266
Concept ID:
C1112256
Disease or Syndrome
Hypertrophic nerve changes
MedGen UID:
322038
Concept ID:
C1832776
Finding
Segmental peripheral demyelination/remyelination
MedGen UID:
335873
Concept ID:
C1843077
Finding
A segmental pattern of demyelination and regeneration (remyelination) affecting peripheral nerves.
Distal sensory impairment
MedGen UID:
335722
Concept ID:
C1847584
Finding
An abnormal reduction in sensation in the distal portions of the extremities.
Onion bulb formation
MedGen UID:
376237
Concept ID:
C1847906
Finding
Repeated episodes of segmental demyelination and remyelination lead to the accumulation of supernumerary Schwann cells around axons, which is referred to as onion bulb formation. This finding affects peripheral nerves.
Decreased nerve conduction velocity
MedGen UID:
347509
Concept ID:
C1857640
Finding
A reduction in the speed at which electrical signals propagate along the axon of a neuron.
Decreased motor nerve conduction velocity
MedGen UID:
388130
Concept ID:
C1858729
Finding
A type of decreased nerve conduction velocity that affects the motor neuron.
Decreased distal sensory nerve action potential
MedGen UID:
870474
Concept ID:
C4024920
Finding
A reduction in the amplitude of sensory nerve action potential in distal nerve segments. This feature is measured by nerve conduction studies.
Distal muscle weakness
MedGen UID:
140883
Concept ID:
C0427065
Finding
Reduced strength of the musculature of the distal extremities.
Distal amyotrophy
MedGen UID:
338530
Concept ID:
C1848736
Disease or Syndrome
Muscular atrophy affecting muscles in the distal portions of the extremities.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Charcot-Marie-Tooth disease type 1C in Orphanet.

Recent clinical studies

Etiology

Guimarães-Costa R, Iancu Ferfoglia R, Leonard-Louis S, Ziegler F, Magy L, Fournier E, Dubourg O, Bouche P, Maisonobe T, Lacour A, Moerman A, Latour P, Stojkovic T
Eur J Neurol 2017 Mar;24(3):530-538. doi: 10.1111/ene.13239. PMID: 28211240
Jerath NU, Shy ME
Muscle Nerve 2017 Dec;56(6):1092-1095. Epub 2017 Apr 29 doi: 10.1002/mus.25600. PMID: 28164329Free PMC Article
Ho AK, Wagstaff JL, Manna PT, Wartosch L, Qamar S, Garman EF, Freund SM, Roberts RC
BMC Biol 2016 Dec 7;14(1):109. doi: 10.1186/s12915-016-0332-8. PMID: 27927196Free PMC Article
Potulska-Chromik A, Sinkiewicz-Darol E, Kostera-Pruszczyk A, Drac H, Kabzińska D, Zakrzewska-Pniewska B, Gołębiowski M, Kochański A
Folia Neuropathol 2012;50(4):369-74. doi: 10.5114/fn.2012.32366. PMID: 23319192
Street VA, Goldy JD, Golden AS, Tempel BL, Bird TD, Chance PF
Am J Hum Genet 2002 Jan;70(1):244-50. Epub 2001 Nov 16 doi: 10.1086/337943. PMID: 11713717Free PMC Article

Diagnosis

Levkova M, Stoyanova M, Benkova-Petrova M, Georgieva M, Angelova L
J Am Assoc Nurse Pract 2022 Aug 1;34(8):1018-1021. doi: 10.1097/JXX.0000000000000745. PMID: 35731603
Turčanová Koprušáková M, Grofik M, Kantorová E, Jungová P, Chandoga J, Kolisek M, Valkovič P, Škorvánek M, Ploski R, Kurča E, Sivák Š
BMC Neurol 2021 Jul 27;21(1):293. doi: 10.1186/s12883-021-02316-3. PMID: 34311727Free PMC Article
Guimarães-Costa R, Iancu Ferfoglia R, Leonard-Louis S, Ziegler F, Magy L, Fournier E, Dubourg O, Bouche P, Maisonobe T, Lacour A, Moerman A, Latour P, Stojkovic T
Eur J Neurol 2017 Mar;24(3):530-538. doi: 10.1111/ene.13239. PMID: 28211240
Jerath NU, Shy ME
Muscle Nerve 2017 Dec;56(6):1092-1095. Epub 2017 Apr 29 doi: 10.1002/mus.25600. PMID: 28164329Free PMC Article
Ho AK, Wagstaff JL, Manna PT, Wartosch L, Qamar S, Garman EF, Freund SM, Roberts RC
BMC Biol 2016 Dec 7;14(1):109. doi: 10.1186/s12915-016-0332-8. PMID: 27927196Free PMC Article

Prognosis

Ho AK, Wagstaff JL, Manna PT, Wartosch L, Qamar S, Garman EF, Freund SM, Roberts RC
BMC Biol 2016 Dec 7;14(1):109. doi: 10.1186/s12915-016-0332-8. PMID: 27927196Free PMC Article
Qin W, Wunderley L, Barrett AL, High S, Woodman PG
Biochem J 2016 Nov 1;473(21):3965-3978. Epub 2016 Aug 31 doi: 10.1042/BCJ20160657. PMID: 27582497Free PMC Article
Potulska-Chromik A, Sinkiewicz-Darol E, Kostera-Pruszczyk A, Drac H, Kabzińska D, Zakrzewska-Pniewska B, Gołębiowski M, Kochański A
Folia Neuropathol 2012;50(4):369-74. doi: 10.5114/fn.2012.32366. PMID: 23319192

Clinical prediction guides

Levkova M, Stoyanova M, Benkova-Petrova M, Georgieva M, Angelova L
J Am Assoc Nurse Pract 2022 Aug 1;34(8):1018-1021. doi: 10.1097/JXX.0000000000000745. PMID: 35731603
Park J, Kim HS, Kwon HM, Kim J, Nam SH, Jung NY, Lee AJ, Jung YH, Kim SB, Chung KW, Choi BO
Genes Genomics 2022 Aug;44(8):1007-1016. Epub 2022 May 24 doi: 10.1007/s13258-022-01253-w. PMID: 35608774
Guimarães-Costa R, Iancu Ferfoglia R, Leonard-Louis S, Ziegler F, Magy L, Fournier E, Dubourg O, Bouche P, Maisonobe T, Lacour A, Moerman A, Latour P, Stojkovic T
Eur J Neurol 2017 Mar;24(3):530-538. doi: 10.1111/ene.13239. PMID: 28211240
Jerath NU, Shy ME
Muscle Nerve 2017 Dec;56(6):1092-1095. Epub 2017 Apr 29 doi: 10.1002/mus.25600. PMID: 28164329Free PMC Article
Li W, Zhu H, Zhao X, Brancho D, Liang Y, Zou Y, Bennett C, Chow CW
Mol Cell Biol 2015 Jul;35(14):2464-78. doi: 10.1128/MCB.00300-15. PMID: 25963657Free PMC Article

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...