Rare diseases · Sign or symptom
Increased CSF protein concentration
HP:0002922
What it means
Increased concentration of protein in the cerebrospinal fluid.
Rare diseases that can present with this53
Very common80–99%
7Common30–79%
34- Acute disseminated encephalomyelitis
- Acute transverse myelitis
- Adult Krabbe disease
- Amoebiasis due to free-living amoebae
- Autoimmune limbic encephalitis
- Bickerstaff brainstem encephalitis
- Charcot-Marie-Tooth disease type 1B
- Cysticercosis
- Encephalitis lethargica
- Familial or sporadic hemiplegic migraine
- Herpes simplex virus encephalitis
- Hyperprolinemia type 2
- Idiopathic uveal effusion syndrome
- IgG4-related pachymeningitis
- Japanese encephalitis
- Kearns-Sayre syndrome
- Listeriosis
- MELAS
- Metachromatic leukodystrophy
- Metachromatic leukodystrophy, adult form
- Metachromatic leukodystrophy, juvenile form
- Metachromatic leukodystrophy, late infantile form
- Miller Fisher syndrome
- Mitochondrial neurogastrointestinal encephalomyopathy
- Multifocal motor neuropathy
- Neutral lipid storage disease with ichthyosis
- New-onset refractory status epilepticus
- Papillary tumor of the pineal region
- Paraneoplastic cerebellar degeneration
- Pineocytoma
- Posttransplant acute limbic encephalitis
- Schilder disease
- Secondary syringomyelia
- Sporadic Creutzfeldt-Jakob disease
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. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Cerebrospinal fluid protein increased · Cerebrospinal fluid with increased protein · Elevated cerebrospinal fluid protein · Elevated csf protein · Hyperproteinorrhachia · Increased CSF protein · Increased protein in csf · Spinal fluid protein elevated
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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.