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Spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 3(SCAN3)

MedGen UID:
1673607
Concept ID:
C5193070
Disease or Syndrome
Synonym: SCAN3
 
Gene (location): COA7 (1p32.3)
 
Monarch Initiative: MONDO:0020770
OMIM®: 618387

Definition

Spinocerebellar ataxia with axonal neuropathy-3 (SCAN3) is an autosomal recessive neuromuscular disorder characterized by onset in the first decade of slowly progressive distal muscle weakness and atrophy and distal sensory impairment due to an axonal peripheral neuropathy. Affected individuals have gait disturbances and sometimes manual dexterity difficulties, as well as cerebellar ataxia associated with cerebellar atrophy on brain imaging. Additional features usually include dysarthria, hyporeflexia, and increased serum creatine kinase. Some patients may have impaired intellectual development (summary by Higuchi et al., 2018). For a discussion of genetic heterogeneity of SCAN, see SCAN1 (607250). [from OMIM]

Clinical features

From HPO
Limb muscle weakness
MedGen UID:
107956
Concept ID:
C0587246
Finding
Reduced strength and weakness of the muscles of the arms and legs.
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).
Hammertoe
MedGen UID:
209712
Concept ID:
C1136179
Anatomical Abnormality
Hyperextension of the metatarsal-phalangeal joint with hyperflexion of the proximal interphalangeal (PIP) joint.
Foot dorsiflexor weakness
MedGen UID:
356163
Concept ID:
C1866141
Finding
Weakness of the muscles responsible for dorsiflexion of the foot, that is, of the movement of the toes towards the shin. The foot dorsiflexors include the tibialis anterior, the extensor hallucis longus, the extensor digitorum longus, and the peroneus tertius muscles.
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).
Dysarthria
MedGen UID:
8510
Concept ID:
C0013362
Mental or Behavioral Dysfunction
Dysarthric speech is a general description referring to a neurological speech disorder characterized by poor articulation. Depending on the involved neurological structures, dysarthria may be further classified as spastic, flaccid, ataxic, hyperkinetic and hypokinetic, or mixed.
Tremor
MedGen UID:
21635
Concept ID:
C0040822
Sign or Symptom
An unintentional, oscillating to-and-fro muscle movement about a joint axis.
Dysmetria
MedGen UID:
68583
Concept ID:
C0234162
Finding
A type of ataxia characterized by the inability to carry out movements with the correct range and motion across the plane of more than one joint related to incorrect estimation of the distances required for targeted movements.
Leukoencephalopathy
MedGen UID:
78722
Concept ID:
C0270612
Disease or Syndrome
This term describes abnormality of the white matter of the cerebrum resulting from damage to the myelin sheaths of nerve cells.
Steppage gait
MedGen UID:
98105
Concept ID:
C0427149
Finding
An abnormal gait pattern that arises from weakness of the pretibial and peroneal muscles due to a lower motor neuron lesion. Affected patients have footdrop and are unable to dorsiflex and evert the foot. The leg is lifted high on walking so that the toes clear the ground, and there may be a slapping noise when the foot strikes the ground again.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Hyporeflexia
MedGen UID:
195967
Concept ID:
C0700078
Finding
Reduction of neurologic reflexes such as the knee-jerk reaction.
Cerebellar atrophy
MedGen UID:
196624
Concept ID:
C0740279
Disease or Syndrome
Cerebellar atrophy is defined as a cerebellum with initially normal structures, in a posterior fossa with normal size, which displays enlarged fissures (interfolial spaces) in comparison to the foliae secondary to loss of tissue. Cerebellar atrophy implies irreversible loss of tissue and result from an ongoing progressive disease until a final stage is reached or a single injury, e.g. an intoxication or infectious event.
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.
Peripheral axonal neuropathy
MedGen UID:
266071
Concept ID:
C1263857
Disease or Syndrome
An abnormality characterized by disruption of the normal functioning of peripheral axons.
Distal sensory impairment
MedGen UID:
335722
Concept ID:
C1847584
Finding
An abnormal reduction in sensation in the distal portions of the extremities.
Demyelinating sensory neuropathy
MedGen UID:
868959
Concept ID:
C4023373
Disease or Syndrome
Demyelination of peripheral sensory nerves.
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.
Elevated circulating creatine kinase concentration
MedGen UID:
69128
Concept ID:
C0241005
Finding
An elevation of the level of the enzyme creatine kinase (also known as creatine phosphokinase (CK; EC 2.7.3.2) in the blood. CK levels can be elevated in a number of clinical disorders such as myocardial infarction, rhabdomyolysis, and muscular dystrophy.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

Kneer K, Straub S, Wittlinger J, Stahl JH, Winter N, Timmann D, Schöls L, Synofzik M, Bender F, Grimm A
J Neurol 2024 May;271(5):2494-2502. Epub 2024 Jan 23 doi: 10.1007/s00415-023-12159-2. PMID: 38261029Free PMC Article
Yahia A, Hamed AAA, Mohamed IN, Elseed MA, Salih MA, El-Sadig SM, Siddig HE, Nasreldien AEM, Abdullah MA, Elzubair M, Omer FY, Bakhiet AM, Abubaker R, Abozar F, Adil R, Emad S, Musallam MA, Eltazi IZM, Omer Z, Malik H, Mohamed MOE, Elhassan AA, Mohamed EOE, Ahmed AKMA, Ahmed EAA, Eltaraifee E, Hussein BK, Abd Allah ASI, Salah L, Nimir M, Tag Elseed OM, Elhassan TEA, Elbashier A, Alfadul ESA, Fadul M, Ali KF, Taha SOMA, Bushara EE, Amin M, Koko M, Ibrahim ME, Ahmed AE, Elsayed LEO, Stevanin G
Eur J Hum Genet 2024 Oct;32(10):1214-1226. Epub 2023 Apr 3 doi: 10.1038/s41431-023-01344-6. PMID: 37012327Free PMC Article
Wiethoff S, Hersheson J, Bettencourt C, Wood NW, Houlden H
J Neurol 2016 Aug;263(8):1503-10. Epub 2016 May 13 doi: 10.1007/s00415-016-8148-6. PMID: 27178001Free PMC Article
Comeaux EQ, van Waardenburg RC
Drug Metab Rev 2014 Nov;46(4):494-507. Epub 2014 Oct 20 doi: 10.3109/03602532.2014.971957. PMID: 25327705
Berciano J, García A, Infante J
Handb Clin Neurol 2013;115:907-32. doi: 10.1016/B978-0-444-52902-2.00051-5. PMID: 23931821

Diagnosis

Kneer K, Straub S, Wittlinger J, Stahl JH, Winter N, Timmann D, Schöls L, Synofzik M, Bender F, Grimm A
J Neurol 2024 May;271(5):2494-2502. Epub 2024 Jan 23 doi: 10.1007/s00415-023-12159-2. PMID: 38261029Free PMC Article
Yahia A, Hamed AAA, Mohamed IN, Elseed MA, Salih MA, El-Sadig SM, Siddig HE, Nasreldien AEM, Abdullah MA, Elzubair M, Omer FY, Bakhiet AM, Abubaker R, Abozar F, Adil R, Emad S, Musallam MA, Eltazi IZM, Omer Z, Malik H, Mohamed MOE, Elhassan AA, Mohamed EOE, Ahmed AKMA, Ahmed EAA, Eltaraifee E, Hussein BK, Abd Allah ASI, Salah L, Nimir M, Tag Elseed OM, Elhassan TEA, Elbashier A, Alfadul ESA, Fadul M, Ali KF, Taha SOMA, Bushara EE, Amin M, Koko M, Ibrahim ME, Ahmed AE, Elsayed LEO, Stevanin G
Eur J Hum Genet 2024 Oct;32(10):1214-1226. Epub 2023 Apr 3 doi: 10.1038/s41431-023-01344-6. PMID: 37012327Free PMC Article
Karuvath RH, Patwari S, Chadaga H
Radiology 2021 Sep;300(3):730-732. doi: 10.1148/radiol.2021203053. PMID: 34424787
Briand MM, Rodrigue X, Lessard I, Mathieu J, Brais B, Côté I, Gagnon C
J Neurol Sci 2019 May 15;400:39-41. Epub 2019 Mar 12 doi: 10.1016/j.jns.2019.03.008. PMID: 30901567
Gücüyener K, Ozgül K, Paternotte C, Erdem H, Prud'homme JF, Ozgüç M, Topaloğlu H
Neuropediatrics 2001 Jun;32(3):142-6. doi: 10.1055/s-2001-16616. PMID: 11521210

Therapy

Comeaux EQ, van Waardenburg RC
Drug Metab Rev 2014 Nov;46(4):494-507. Epub 2014 Oct 20 doi: 10.3109/03602532.2014.971957. PMID: 25327705

Prognosis

Vermeer S, Meijer RP, Pijl BJ, Timmermans J, Cruysberg JR, Bos MM, Schelhaas HJ, van de Warrenburg BP, Knoers NV, Scheffer H, Kremer B
Neurogenetics 2008 Jul;9(3):207-14. Epub 2008 May 9 doi: 10.1007/s10048-008-0131-7. PMID: 18465152Free PMC Article

Clinical prediction guides

Kneer K, Straub S, Wittlinger J, Stahl JH, Winter N, Timmann D, Schöls L, Synofzik M, Bender F, Grimm A
J Neurol 2024 May;271(5):2494-2502. Epub 2024 Jan 23 doi: 10.1007/s00415-023-12159-2. PMID: 38261029Free PMC Article
van Minkelen R, Guitart M, Escofet C, Yoon G, Elfferich P, Bolman GM, van der Helm R, van de Graaf R, van den Ouweland AM
BMC Med Genet 2015 Aug 19;16:61. doi: 10.1186/s12881-015-0213-y. PMID: 26285866Free PMC Article
Comeaux EQ, Cuya SM, Kojima K, Jafari N, Wanzeck KC, Mobley JA, Bjornsti MA, van Waardenburg RC
J Biol Chem 2015 Mar 6;290(10):6203-14. Epub 2015 Jan 21 doi: 10.1074/jbc.M114.635284. PMID: 25609251Free PMC Article
Vermeer S, Meijer RP, Pijl BJ, Timmermans J, Cruysberg JR, Bos MM, Schelhaas HJ, van de Warrenburg BP, Knoers NV, Scheffer H, Kremer B
Neurogenetics 2008 Jul;9(3):207-14. Epub 2008 May 9 doi: 10.1007/s10048-008-0131-7. PMID: 18465152Free PMC Article
Bouhlal Y, El-Euch-Fayeche G, Amouri R, Hentati F
Acta Myol 2005 Oct;24(2):155-61. PMID: 16550933

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