Rare diseases · Sign or symptom
Babinski sign
HP:0003487
What it means
Upturning of the big toe (and sometimes fanning of the other toes) in response to stimulation of the sole of the foot. If the Babinski sign is present it can indicate damage to the corticospinal tract.
A positive Babinski sign can indicate damage to the corticospinal tract.
Rare diseases that can present with this155
Very common80–99%
32- Ataxia with vitamin E deficiency
- Autosomal dominant spastic paraplegia type 19
- Autosomal dominant spastic paraplegia type 29
- Autosomal dominant spastic paraplegia type 3
- Autosomal dominant spastic paraplegia type 36
- Autosomal dominant spastic paraplegia type 38
- Autosomal dominant spastic paraplegia type 42
- Autosomal dominant spastic paraplegia type 6
- Autosomal dominant spastic paraplegia type 73
- Autosomal dominant spastic paraplegia type 8
- Autosomal recessive cerebellar ataxia with late-onset spasticity
- Autosomal recessive spastic ataxia-optic atrophy-dysarthria syndrome
- Autosomal recessive spastic paraplegia type 14
- Autosomal recessive spastic paraplegia type 27
- Autosomal recessive spastic paraplegia type 28
- Autosomal recessive spastic paraplegia type 35
- Autosomal recessive spastic paraplegia type 43
- Autosomal recessive spastic paraplegia type 45
- Autosomal recessive spastic paraplegia type 46
- Autosomal recessive spastic paraplegia type 56
- Autosomal recessive spastic paraplegia type 57
- Autosomal recessive spastic paraplegia type 5A
- Autosomal recessive spastic paraplegia type 75
- Autosomal recessive spastic paraplegia type 76
- Autosomal recessive spastic paraplegia type 78
- Autosomal recessive spastic paraplegia type 9B
- Autosomal spastic paraplegia type 30
- Friedreich ataxia
- Kufor-Rakeb syndrome
- Mitochondrial membrane protein-associated neurodegeneration
- Primary lateral sclerosis
- Spastic paraplegia type 2
Common30–79%
47- Adrenomyeloneuropathy
- Adult Krabbe disease
- Adult-onset autosomal dominant leukodystrophy
- Alexander disease type II
- Amyotrophic lateral sclerosis
- Amyotrophic lateral sclerosis type 4
- Autosomal dominant Charcot-Marie-Tooth disease type 2Z
- Autosomal dominant dopa-responsive dystonia
- Autosomal dominant spastic ataxia type 1
- Autosomal dominant spastic paraplegia type 10
- Autosomal dominant spastic paraplegia type 12
- Autosomal dominant spastic paraplegia type 13
- Autosomal dominant spastic paraplegia type 17
- Autosomal dominant spastic paraplegia type 37
- Autosomal dominant spastic paraplegia type 4
- Autosomal dominant spastic paraplegia type 9A
- Autosomal dominant spastic paraplegia type 9B
- Autosomal recessive ataxia, Beauce type
- Autosomal recessive cerebellar ataxia due to CWF19L1 deficiency
- Autosomal recessive complex spastic paraplegia due to Kennedy pathway dysfunction
- Autosomal recessive dopa-responsive dystonia
- Autosomal recessive spastic ataxia of Charlevoix-Saguenay
- Autosomal recessive spastic paraplegia type 20
- Autosomal recessive spastic paraplegia type 26
- Autosomal recessive spastic paraplegia type 32
- Autosomal recessive spastic paraplegia type 39
- Autosomal recessive spastic paraplegia type 55
- Autosomal recessive spastic paraplegia type 67
- Autosomal recessive spastic paraplegia type 71
- Autosomal recessive spastic paraplegia type 77
- Autosomal spastic paraplegia type 18
- Autosomal spastic paraplegia type 58
- Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome
- Cerebrotendinous xanthomatosis
- Childhood-onset autosomal recessive slowly progressive spinocerebellar ataxia
- Childhood-onset spasticity with hyperglycinemia
- CLN12 disease
- DPM3-CDG
- Dystonia-parkinsonism-hypermanganesemia syndrome
- Familial infantile bilateral striatal necrosis
- Infantile-onset autosomal recessive nonprogressive cerebellar ataxia
- Inherited Creutzfeldt-Jakob disease
- Isolated succinate-CoQ reductase deficiency
- Kjellin syndrome
- Leukoencephalopathy-spondyloepimetaphyseal dysplasia syndrome
- Leukoencephalopathy with bilateral anterior temporal lobe cysts
- Leukoencephalopathy with brain stem and spinal cord involvement-high lactate syndrome
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 1 disease where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Extensor plantar reflexes · Extensor plantar response · Extensor plantar responses · Positive Babinski sign
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.