U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ulnar claw

MedGen UID:
871311
Concept ID:
C4025799
Congenital Abnormality
HPO: HP:0001178

Definition

An abnormal hand position characterized by hyperextension of the fourth and fifth fingers at the metacarpophalangeal joints and flexion of the interphalangeal joints of the same fingers such that they are curled towards the palm. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUlnar claw

Conditions with this feature

Dejerine-Sottas disease
MedGen UID:
3710
Concept ID:
C0011195
Disease or Syndrome
Dejerine-Sottas neuropathy is a demyelinating peripheral neuropathy with onset in infancy. It can show autosomal dominant or recessive inheritance. Affected individuals have delayed motor development due to severe distal motor and sensory impairment, resulting in difficulties in gait. Some patients have generalized hypotonia in infancy. Other features may include pes cavus, scoliosis, and sensory ataxia. Nerve conduction velocities are severely decreased (sometimes less than 10 m/s), and sural nerve biopsy shows severe loss of myelinated fibers (summary by Baets et al., 2011).
Charcot-Marie-Tooth disease, type IA
MedGen UID:
75727
Concept ID:
C0270911
Disease or Syndrome
For a general phenotypic description and a discussion of genetic heterogeneity of Charcot-Marie-Tooth disease type 1, see CMT1B (118200). CMT1A is the most common form of CMT. The average age of onset of clinical symptoms is 12.2 +/- 7.3 years. Slow nerve conduction velocity (NCV) less than 38 m/s is highly diagnostic and is a 100% penetrant phenotype independent of age (Lupski et al., 1991, 1992).
Charcot-Marie-Tooth disease type 1B
MedGen UID:
124377
Concept ID:
C0270912
Disease or Syndrome
Charcot-Marie-Tooth disease is a sensorineural peripheral polyneuropathy. Affecting approximately 1 in 2,500 individuals, Charcot-Marie-Tooth disease is the most common inherited disorder of the peripheral nervous system (Skre, 1974). Autosomal dominant, autosomal recessive, and X-linked forms have been recognized. Classification On the basis of electrophysiologic properties and histopathology, CMT has been divided into primary peripheral demyelinating (type 1, or HMSNI) and primary peripheral axonal (type 2, or HMSNII) neuropathies. The demyelinating neuropathies classified as CMT type 1 are characterized by severely reduced motor NCVs (less than 38 m/s) and segmental demyelination and remyelination with onion bulb formations on nerve biopsy. The axonal neuropathies classified as CMT type 2 are characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy (see CMT2A1; 118210). Distal hereditary motor neuropathy (dHMN) (see 158590), or spinal CMT, is characterized by exclusive motor involvement and sparing of sensory nerves (Pareyson, 1999). McAlpine (1989) proposed that the forms of CMT with very slow nerve conduction be given the gene symbol CMT1A (118220) and CMT1B, CMT1A being the gene on chromosome 17 and CMT1B being the gene on chromosome 1. CMT2 was the proposed symbol for the autosomal locus responsible for the moderately slow nerve conduction form of the disease (axonal). For a phenotypic description and discussion of genetic heterogeneity of the various subtypes of CMT, see CMTX1 (302800), CMT2A1 (118210), CMT3 (DSS; 145900), CMT4A (214400), and CMTDIB (606482). Genetic Heterogeneity of Autosomal Dominant Demyelinating CMT1 Autosomal dominant demyelinating CMT1 is a genetically heterogeneous disorder and can be caused by mutations in different genes; see CMT1A (118220), CMT1C (601098), CMT1D (607678), CMT1E (607734), CMT1F (607734), CMT1G (618279), CMT1H (619764), CMT1I (619742), and CMT1J (620111). See also 608236 for a related phenotype characterized by isolated slowed nerve conduction velocities (NCVs).
Charcot-Marie-Tooth disease recessive intermediate A
MedGen UID:
334012
Concept ID:
C1842197
Disease or Syndrome
GDAP1-related hereditary motor and sensory neuropathy (GDAP1-HMSN) is a peripheral neuropathy (also known as a subtype of Charcot-Marie-Tooth disease) that typically affects the lower extremities earlier and more severely than the upper extremities. As the neuropathy progresses, the distal upper extremities also become severely affected. Proximal muscles can also become weak. Age at onset ranges from infancy to early childhood. In most cases, disease progression causes disabilities within the first or second decade of life. At the end of the second decade, most individuals are wheelchair bound. Disease progression varies considerably even within the same family. The neuropathy can be either of the demyelinating type with reduced nerve conduction velocities or the axonal type with normal nerve conduction velocities. Vocal cord paresis is common. Intelligence is normal. Life expectancy is usually normal, but on occasion may be reduced because of secondary complications.
Charcot-Marie-Tooth disease, axonal, with vocal cord paresis, autosomal recessive
MedGen UID:
375113
Concept ID:
C1843183
Disease or Syndrome
Charcot-Marie-Tooth disease type 2E
MedGen UID:
375127
Concept ID:
C1843225
Disease or Syndrome
A form of axonal Charcot-Marie-Tooth disease a peripheral sensorimotor neuropathy. Onset is in the first to sixth decade with a gait anomaly and a leg weakness that reaches the arms secondarily. Tendon reflexes are reduced or absent and after years all patients have a pes cavus. Other signs may be present including hearing loss and postural tremor.
Charcot-Marie-Tooth disease axonal type 2F
MedGen UID:
335784
Concept ID:
C1847823
Disease or Syndrome
A form of axonal Charcot-Marie-Tooth disease, a peripheral sensorimotor neuropathy with symmetric weakness primarily occurring in the lower limbs and reaching the arms only after 5 to 10 years, occasional and predominantly distal sensory loss and reduced tendon reflexes. Presents with gait anomaly between the first and sixth decade and early onset is generally associated to a more severe phenotype that may include foot drop.
Charcot-Marie-Tooth disease type 4B2
MedGen UID:
346869
Concept ID:
C1858278
Disease or Syndrome
Autosomal recessive Charcot-Marie-Tooth disease type 4B2 (CMT4B2) is a demyelinating hereditary motor and sensory neuropathy characterized by abnormal folding of myelin sheaths. CMT4B1 (601382) is a clinically similar disorder caused by mutation in the MTMR2 gene (603557) on 11q22. For a phenotypic description and a discussion of genetic heterogeneity of autosomal recessive demyelinating CMT, see CMT4A (214400).
Charcot-Marie-Tooth disease type 4A
MedGen UID:
347821
Concept ID:
C1859198
Disease or Syndrome
GDAP1-related hereditary motor and sensory neuropathy (GDAP1-HMSN) is a peripheral neuropathy (also known as a subtype of Charcot-Marie-Tooth disease) that typically affects the lower extremities earlier and more severely than the upper extremities. As the neuropathy progresses, the distal upper extremities also become severely affected. Proximal muscles can also become weak. Age at onset ranges from infancy to early childhood. In most cases, disease progression causes disabilities within the first or second decade of life. At the end of the second decade, most individuals are wheelchair bound. Disease progression varies considerably even within the same family. The neuropathy can be either of the demyelinating type with reduced nerve conduction velocities or the axonal type with normal nerve conduction velocities. Vocal cord paresis is common. Intelligence is normal. Life expectancy is usually normal, but on occasion may be reduced because of secondary complications.
Charcot-Marie-Tooth disease, axonal, type 2EE
MedGen UID:
1677426
Concept ID:
C5193076
Disease or Syndrome
Charcot-Marie-Tooth disease type 2EE (CMT2EE) is an autosomal recessive sensorimotor peripheral axonal neuropathy with onset in the first or second decades of life. The disorder primarily affects the lower limbs and is slowly progressive, sometimes resulting in loss of ambulation, with later onset of upper limb involvement. There is significant distal muscle weakness and atrophy, usually with foot or hand deformities. Skeletal muscle biopsy shows findings of disturbed mitochondrial maintenance. Cognition is unaffected, and chronic liver disease is absent (summary by Baumann et al., 2019). For a phenotypic description and a discussion of genetic heterogeneity of axonal CMT type 2, see CMT2A (118210).

Professional guidelines

PubMed

Mendiratta V, Khan A, Jain A
Indian J Lepr 2006 Jul-Sep;78(3):261-7. PMID: 17120509

Recent clinical studies

Etiology

Chang CH, Wang JP, Huang YC, Yin CY, Chen KH, Huang HK
BMC Surg 2024 Oct 3;24(1):284. doi: 10.1186/s12893-024-02583-0. PMID: 39363274Free PMC Article
Singh AK, Dixit P, Singh V, Vardhan H
J Plast Reconstr Aesthet Surg 2022 Sep;75(9):3279-3284. Epub 2022 Apr 27 doi: 10.1016/j.bjps.2022.04.054. PMID: 35672246
Sette CS, Wachholz PA, Masuda PY, Figueira RB, Filho MC, Soares CT, Barreto JA
Rev Soc Bras Med Trop 2015 Mar-Apr;48(2):228-30. doi: 10.1590/0037-8682-0259-2014. PMID: 25992943
Taylor NL, Raj AD, Dick HM, Solomon S
J Hand Surg Am 2004 Jul;29(4):595-604. doi: 10.1016/j.jhsa.2004.03.006. PMID: 15249082
Sahoo A, Singh PC, Pattnaik S, Singh N
Indian J Lepr 2002 Apr-Jun;74(2):137-43. PMID: 12708732

Diagnosis

Chang CH, Wang JP, Huang YC, Yin CY, Chen KH, Huang HK
BMC Surg 2024 Oct 3;24(1):284. doi: 10.1186/s12893-024-02583-0. PMID: 39363274Free PMC Article
Sette CS, Wachholz PA, Masuda PY, Figueira RB, Filho MC, Soares CT, Barreto JA
Rev Soc Bras Med Trop 2015 Mar-Apr;48(2):228-30. doi: 10.1590/0037-8682-0259-2014. PMID: 25992943
Neiman R, Maiocco B, Deeney VF
J Pediatr Orthop 1998 Sep-Oct;18(5):683-5. doi: 10.1097/00004694-199809000-00026. PMID: 9746426

Therapy

Brown PW
J Bone Joint Surg Am 1970 Jul;52(5):868-77. PMID: 5479477

Prognosis

Chang CH, Wang JP, Huang YC, Yin CY, Chen KH, Huang HK
BMC Surg 2024 Oct 3;24(1):284. doi: 10.1186/s12893-024-02583-0. PMID: 39363274Free PMC Article
Taylor NL, Raj AD, Dick HM, Solomon S
J Hand Surg Am 2004 Jul;29(4):595-604. doi: 10.1016/j.jhsa.2004.03.006. PMID: 15249082
Sahoo A, Singh PC, Pattnaik S, Singh N
Indian J Lepr 2002 Apr-Jun;74(2):137-43. PMID: 12708732

Clinical prediction guides

Shah A
J Hand Surg Br 1984 Jun;9(2):131-3. PMID: 6747411

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...