Birk-Barel syndrome

MeSH: C567357ORPHA: 166108

Overview

Human disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Birk-Barel syndrome, sourced from HPO and Orphanet clinical annotations.

Open mouthDolichocephalyBroad philtrumShort philtrumHypomimic faceNarrow foreheadMicrognathiaProtruding earNarrow nasal bridgeBroad nasal tipHyperactivitySacral dimpleGlobal developmental delayGeneralized hypotoniaNeonatal hypotoniaDysphoniaDysphagiaHighly arched eyebrowHigh, narrow palateTented upper lip vermilionIncisor macrodontiaFeeding difficultiesThick vermilion borderFatigable weakness of skeletal musclesFatiguable weakness of proximal limb musclesNasogastric tube feedingAreflexiaTongue fasciculationsLimited elbow flexion/extensionCongenital finger flexion contracturesMotor axonal neuropathySpinal muscular atrophyFoot joint contracture

Classification & Codes

MeSH Code

C567357

Orphanet Code

ORPHA:166108
Birk-Barel syndrome
MeSHC567357
OrphanetORPHA:166108
Treatments0 drug(s)
Symptoms on record33 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO