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Esclerose lateral amiotrófica juvenil
ORPHA:300605CID-10 · G12.2PCDT · SUSDOENÇA RARA

A Esclerose Lateral Amiotrófica Juvenil (JALS) é uma doença muito rara e grave que afeta os nervos que controlam os movimentos. Ela é caracterizada pela perda progressiva desses nervos, tanto os que vêm do cérebro quanto os da medula espinhal, o que causa rigidez muscular no rosto, dificuldade para falar (disartria) e problemas para andar. Os primeiros sintomas surgem antes dos 25 anos de idade.

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Introdução

O que você precisa saber de cara

📋

A Esclerose Lateral Amiotrófica Juvenil (JALS) é uma doença muito rara e grave que afeta os nervos que controlam os movimentos. Ela é caracterizada pela perda progressiva desses nervos, tanto os que vêm do cérebro quanto os da medula espinhal, o que causa rigidez muscular no rosto, dificuldade para falar (disartria) e problemas para andar. Os primeiros sintomas surgem antes dos 25 anos de idade.

Publicações científicas
137 artigos
Último publicado: 2026 Mar 12

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Childhood
🏥
SUS: Cobertura parcialScore: 65%
PCDT disponível2 medicamentos CEAFCID-10: G12.2
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
22 sintomas
💪
Músculos
19 sintomas
🦴
Ossos e articulações
4 sintomas
📏
Crescimento
3 sintomas
🫃
Digestivo
1 sintomas
👁️
Olhos
1 sintomas

+ 25 sintomas em outras categorias

Características mais comuns

90%prev.
Espasticidade de membro superior
Muito frequente (99-80%)
90%prev.
Amiotrofia distal
Muito frequente (99-80%)
90%prev.
Fraqueza muscular
Muito frequente (99-80%)
90%prev.
Esclerose lateral amiotrófica
Muito frequente (99-80%)
90%prev.
Atrofia do músculo esquelético
Muito frequente (99-80%)
90%prev.
Espasticidade do membro inferior
Muito frequente (99-80%)
77sintomas
Muito frequente (6)
Frequente (15)
Ocasional (25)
Sem dados (31)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 77 características clínicas mais associadas, ordenadas por frequência.

Espasticidade de membro superiorUpper limb spasticity
Muito frequente (99-80%)90%
Amiotrofia distalDistal amyotrophy
Muito frequente (99-80%)90%
Fraqueza muscularMuscle weakness
Muito frequente (99-80%)90%
Esclerose lateral amiotróficaAmyotrophic lateral sclerosis
Muito frequente (99-80%)90%
Atrofia do músculo esqueléticoSkeletal muscle atrophy
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico137PubMed
Últimos 10 anos69publicações
Pico20169 papers
Linha do tempo
2026Hoje · 2026🧪 2015Primeiro ensaio clínico📈 2016Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

5 genes identificados com associação a esta condição. Padrão de herança: Autosomal recessive.

ALS2AlsinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

May act as a GTPase regulator. Controls survival and growth of spinal motoneurons (By similarity)

LOCALIZAÇÃO

VIAS BIOLÓGICAS (2)
RAC1 GTPase cycleRAB GEFs exchange GTP for GDP on RABs
MECANISMO DE DOENÇA

Amyotrophic lateral sclerosis 2

A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.

OUTRAS DOENÇAS (4)
infantile-onset ascending hereditary spastic paralysisjuvenile primary lateral sclerosisamyotrophic lateral sclerosis type 2, juvenilejuvenile amyotrophic lateral sclerosis
HGNC:443UniProt:Q96Q42
FUSRNA-binding protein FUSDisease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

DNA/RNA-binding protein that plays a role in various cellular processes such as transcription regulation, RNA splicing, RNA transport, DNA repair and damage response (PubMed:27731383). Binds to ssRNA containing the consensus sequence 5'-AGGUAA-3' (PubMed:21256132). Binds to nascent pre-mRNAs and acts as a molecular mediator between RNA polymerase II and U1 small nuclear ribonucleoprotein thereby coupling transcription and splicing (PubMed:26124092). Also binds its own pre-mRNA and autoregulates

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (4)
mRNA Splicing - Major PathwaymRNA PolyadenylationProcessing of Capped Intron-Containing Pre-mRNADengue Virus-Host Interactions
EXPRESSÃO TECIDUAL(Ubíquo)
Ovário
193.2 TPM
Cérebro - Hemisfério cerebelar
173.6 TPM
Cerebelo
159.0 TPM
Cervix Ectocervix
155.5 TPM
Cervix Endocervix
152.7 TPM
OUTRAS DOENÇAS (7)
tremor, hereditary essential, 4amyotrophic lateral sclerosis type 6myxoid/round cell liposarcomajuvenile amyotrophic lateral sclerosis
HGNC:4010UniProt:P35637
SPTLC1Serine palmitoyltransferase 1Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the serine palmitoyltransferase multisubunit enzyme (SPT) that catalyzes the initial and rate-limiting step in sphingolipid biosynthesis by condensing L-serine and activated acyl-CoA (most commonly palmitoyl-CoA) to form long-chain bases. The SPT complex is also composed of SPTLC2 or SPTLC3 and SPTSSA or SPTSSB. Within this complex, the heterodimer with SPTLC2 or SPTLC3 forms the catalytic core (PubMed:19416851, PubMed:33558762, PubMed:36170811). The composition of the serine palmit

LOCALIZAÇÃO

Endoplasmic reticulum membrane

VIAS BIOLÓGICAS (1)
Sphingolipid de novo biosynthesis
MECANISMO DE DOENÇA

Amyotrophic lateral sclerosis 27, juvenile

A form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. ALS27 is an autosomal dominant form manifesting as toe walking and gait abnormalities in early childhood.

EXPRESSÃO TECIDUAL(Ubíquo)
Fibroblastos
55.6 TPM
Esôfago - Mucosa
37.6 TPM
Cervix Ectocervix
36.9 TPM
Ovário
36.8 TPM
Vagina
36.5 TPM
OUTRAS DOENÇAS (4)
neuropathy, hereditary sensory and autonomic, type 1Aamyotrophic lateral sclerosis 27, juvenilejuvenile amyotrophic lateral sclerosishereditary sensory and autonomic neuropathy type 1
HGNC:11277UniProt:O15269
SPG11SpatacsinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

May play a role in neurite plasticity by maintaining cytoskeleton stability and regulating synaptic vesicle transport

LOCALIZAÇÃO

Cytoplasm, cytosolNucleusCell projection, axonCell projection, dendrite

MECANISMO DE DOENÇA

Spastic paraplegia 11, autosomal recessive

A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
33.0 TPM
Tireoide
29.3 TPM
Baço
26.6 TPM
Fibroblastos
23.3 TPM
Pulmão
22.7 TPM
OUTRAS DOENÇAS (4)
amyotrophic lateral sclerosis type 5Charcot-Marie-Tooth disease axonal type 2Xhereditary spastic paraplegia 11juvenile amyotrophic lateral sclerosis
HGNC:11226UniProt:Q96JI7
SIGMAR1Sigma non-opioid intracellular receptor 1Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Functions in lipid transport from the endoplasmic reticulum and is involved in a wide array of cellular functions probably through regulation of the biogenesis of lipid microdomains at the plasma membrane. Involved in the regulation of different receptors it plays a role in BDNF signaling and EGF signaling. Also regulates ion channels like the potassium channel and could modulate neurotransmitter release. Plays a role in calcium signaling through modulation together with ANK2 of the ITP3R-depend

LOCALIZAÇÃO

Nucleus inner membraneNucleus outer membraneNucleus envelopeCytoplasmic vesicleEndoplasmic reticulum membraneMembraneLipid dropletCell junctionCell membraneCell projection, growth conePostsynaptic density membrane

VIAS BIOLÓGICAS (1)
Potential therapeutics for SARS
MECANISMO DE DOENÇA

Amyotrophic lateral sclerosis 16, juvenile

A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.

EXPRESSÃO TECIDUAL(Ubíquo)
Fibroblastos
143.9 TPM
Fígado
106.6 TPM
Cervix Ectocervix
101.1 TPM
Cervix Endocervix
93.5 TPM
Útero
86.8 TPM
OUTRAS DOENÇAS (3)
amyotrophic lateral sclerosis type 16autosomal recessive distal spinal muscular atrophy 2juvenile amyotrophic lateral sclerosis
HGNC:8157UniProt:Q99720

Variantes genéticas (ClinVar)

390 variantes patogênicas registradas no ClinVar.

🧬 ALS2: NM_020919.4(ALS2):c.4515G>A (p.Trp1505Ter) ()
🧬 ALS2: NM_020919.4(ALS2):c.1082_1083del (p.Asp360_Ser361insTer) ()
🧬 ALS2: NM_020919.4(ALS2):c.3004C>T (p.Gln1002Ter) ()
🧬 ALS2: NM_020919.4(ALS2):c.3307C>T (p.His1103Tyr) ()
🧬 ALS2: GRCh37/hg19 2q31.3-33.1(chr2:181362315-202911548)x1 ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 15 variantes classificadas pelo ClinVar.

12
3
Patogênica (80.0%)
VUS (20.0%)
VARIANTES MAIS SIGNIFICATIVAS
ALS2: NM_020919.4(ALS2):c.4270C>T (p.Gln1424Ter) [Pathogenic]
SPG11: NM_025137.4(SPG11):c.4432C>T (p.Gln1478Ter) [Pathogenic]
PLEKHG5: NM_020631.6(PLEKHG5):c.1889C>A (p.Pro630His) [Likely pathogenic]
PLEKHG5: NM_020631.6(PLEKHG5):c.1417C>T (p.Gln473Ter) [Pathogenic]
SYNE1: NM_182961.4(SYNE1):c.23524C>T (p.Arg7842Ter) [Pathogenic]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
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Onde tratar no SUS

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🇧🇷 Atendimento SUS — Esclerose lateral amiotrófica juvenil

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Publicações mais relevantes

Timeline de publicações
70 papers (10 anos)
#1

An Unusual Presentation of Juvenile Amyotrophic Lateral Sclerosis with Superoxide Dismutase 1 Mutation: Subacute Bulbar Palsy With Asymmetric Limb Weakness.

Annals of Indian Academy of Neurology2026 Mar 12
#2

SIGMAR1 gene-related neuromuscular disorders - what do we know?

Neurologia i neurochirurgia polska2026

Distal hereditary motor neuropathies (dHMNs) are a clinically and genetically diverse group of rare neuromuscular disorders characterized by progressive distal muscle weakness and atrophy, often with early onset and sparing of sensory function. One subtype, Jerash-type dHMN (dHMNJ), is caused by biallelic mutations in the SIGMAR1 gene and presents with pyramidal signs in addition to distal weakness. A literature review was conducted by searches of the MEDLINE and PubMed databases using selected terms. Relevant original articles, case reports, case series, and reviews were selected as data sources. SIGMAR1-related disorders (SIGMAR1-RD) encompass a broad clinical spectrum including dHMN and juvenile amyotrophic lateral sclerosis (ALS) phenotypes. The Sigma-1 receptor plays a key role in cellular stress responses, ER-mitochondria interaction, and neuronal survival. Clinical presentation often includes distal muscle weakness and atrophy with pyramidal signs. We present a 12-year-old boy with distal muscle weakness, foot drop, and pyramidal signs. Genetic testing identified a homozygous c.247T > C (p.Phe83Leu) SIGMAR1 variant, previously classified as a variant of uncertain significance (VUS). This article supports the pathogenicity of the c.247T > C (p.Phe83Leu) SIGMAR1 variant and underlines the need for broader genetic testing in hereditary motor neuropathies.

#3

A novel heterozygous variant of FUS gene associated with juvenile ALS and premature tremor onset: a case report.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration2026 Feb

Juvenile amyotrophic lateral sclerosis (JALS) is neurodegenerative disease of the upper and lower motor neurons of rare incidence. Although fused in sarcoma (FUS) mutations in JALS patients have been associated with movement disorders, here we described the case of a young girl with very early onset of tremor, years before commencement of weakness; once symptoms of JALS were stablished, a typical rapid disease progression from spinal to bulbar symptoms were noticed. Genetic testing revealed a novel mutation in FUS gene causative of a protein dysfunction. This case emphasizes the fact that some mutations within the FUS in JALS patients may produce a symptom onset with tremor.

#4

SYNE1 Deficiency Manifesting Primarily With Motor Neuron Disease.

Neurology. Genetics2025 Dec

SYNE1 deficiency is an autosomal recessive disorder with a broad phenotypic spectrum, most commonly presenting as adult-onset cerebellar ataxia with or without motor neuron dysfunction. We aimed to expand this spectrum by describing the clinical and genetic findings in 2 unrelated families with early-onset motor neuron disease and virtually no cerebellar signs over time. We performed detailed clinical, neurophysiologic, and genetic studies of 2 unrelated families with juvenile amyotrophic lateral sclerosis (ALS) and biallelic variants in SYNE1. The phenotypes of both families showed onset of symptoms in childhood or adolescence, with signs of upper and lower motor neuron dysfunction in multiple territories suggestive of juvenile ALS. Patients developed progressive muscle weakness, eventually leading to respiratory distress and bulbar signs. Whole-exome sequencing identified SYNE1 biallelic truncating variants in both families: a homozygous nonsense variant, c.23131C>T (p.Gln7711*), in Family 1, and a novel homozygous splice-site variant, c.17851-1G>A, in Family 2. Notably, mild or no cerebellar manifestations were observed during the follow-up. This report highlights a novel phenotype of SYNE1 deficiency characterized by early-onset motor neuron disease and virtually no cerebellar manifestations, broadening the phenotypic spectrum of this complex neurodegenerative disease. These findings support investigating SYNE1 variants in juvenile ALS and including SYNE1 in motor neuron disease gene panels.

#5

Pathology of three ALS patients with FUS variants, including one likely benign Q23L variant lacking FUS inclusions.

Human molecular genetics2025 Sep 03

Fused in sarcoma (FUS) is an RNA-binding protein implicated in juvenile amyotrophic lateral sclerosis (ALS). Mutations in the FUS gene, particularly those affecting the nuclear localization signal (NLS), impair nuclear import and lead to cytoplasmic accumulation of FUS inclusions in motor neurons. However, the pathological and clinical significance of FUS variants outside the NLS remains less understood. Here, we describe clinical and histopathological findings from three ALS patients carrying FUS variants: two with NLS-region variants (R495X and P525L), and one with a variant in the N-terminal region outside the NLS (Q23L). The patients carrying NLS variants presented with aggressive, juvenile-onset spinal and bulbar ALS, characterized primarily by lower motor neuron involvement and rapid disease progression. In contrast, the Q23L patient exhibited a slowly progressive disease course, with predominantly upper motor neuron signs. Neuropathological analysis revealed cytoplasmic FUS inclusions in motor neurons of patients with NLS variants, consistent with typical FUS pathology. In contrast, the Q23L patient lacked FUS inclusions and instead displayed pTDP-43 pathology in the hippocampus, neocortex (including the motor cortex), nucleus olivaris, lentiform nucleus, striatum, and some lower motor neurons. Taken together, these results suggest that Q23L is most likely a benign variant. As antisense oligonucleotides (ASOs) targeting FUS are currently being explored in clinical trials, further neuropathological investigations are needed to determine whether ASO-mediated FUS silencing would be effective for patients carrying FUS variants outside the NLS region.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC72 artigos no totalmostrando 69

2026

An Unusual Presentation of Juvenile Amyotrophic Lateral Sclerosis with Superoxide Dismutase 1 Mutation: Subacute Bulbar Palsy With Asymmetric Limb Weakness.

Annals of Indian Academy of Neurology
2025

Gne deletion in adult mice can cause thrombocytopenia, anemia, myopathy, bleeding, and death.

Journal of neuromuscular diseases
2026

SIGMAR1 gene-related neuromuscular disorders - what do we know?

Neurologia i neurochirurgia polska
2025

SYNE1 Deficiency Manifesting Primarily With Motor Neuron Disease.

Neurology. Genetics
2025

Acquired hemophilia a in a female with minimal change disease and hypothyroidism: a rare case report.

Annals of medicine and surgery (2012)
2025

Ataxia and oculomotor apraxia caused by a large-scale deletion in the senataxin gene.

Journal of applied genetics
2025

Pathology of three ALS patients with FUS variants, including one likely benign Q23L variant lacking FUS inclusions.

Human molecular genetics
2026

A novel heterozygous variant of FUS gene associated with juvenile ALS and premature tremor onset: a case report.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2025

A case of rapidly progressive juvenile amyotrophic lateral sclerosis associated with a pathogenetic heterozygous de novo variant in the FUS gene.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

A novel SIGMAR1 missense mutation leads to distal hereditary motor neuropathy phenotype mimicking juvenile ALS: a case report of China.

Frontiers in genetics
2025

Multiomics Approach Reveal Novel Insights in FUS Driven Juvenile Amyotrophic Lateral Sclerosis: A Family Quartet Analysis.

Annals of neurosciences
2024

Genetic and functional analyses of SPTLC1 in juvenile amyotrophic lateral sclerosis.

Journal of neurology
2025

Phenotype and Genotype of Children with ALS2 gene-Related Disorder.

Neuropediatrics
2024

Juvenile Amyotrophic Lateral Sclerosis: A Case Report of a Rare and Aggressive Presentation in a 22-Year-Old Filipino Male.

Cureus
2024

Serine Palmitoyltransferase (SPT)-related Neurodegenerative and Neurodevelopmental Disorders.

Journal of neuromuscular diseases
2024

Modeling sporadic juvenile ALS in iPSC-derived motor neurons explores the pathogenesis of FUSR503fs mutation.

Frontiers in cellular neuroscience
2024

Distal hereditary motor neuropathies.

Revue neurologique
2024

Clinical and Genetic Aspects of Juvenile Amyotrophic Lateral Sclerosis: A Promising Era Emerges.

Genes
2024

Recurrent de-novo gain-of-function mutation in SPTLC2 confirms dysregulated sphingolipid production to cause juvenile amyotrophic lateral sclerosis.

Journal of neurology, neurosurgery, and psychiatry
2024

Association of TRMT2B gene variants with juvenile amyotrophic lateral sclerosis.

Frontiers of medicine
2024

A brief report on juvenile amyotrophic lateral sclerosis cases in the United States National ALS Registry: 2010-2018.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2023

Thirty-Year Follow-Up of Early Onset Amyotrophic Lateral Sclerosis with a Pathogenic Variant in SPTLC1.

Case reports in neurology
2023

SPTLC1 p.Leu38Arg, a novel mutation associated with childhood ALS.

Biochimica et biophysica acta. Molecular and cell biology of lipids
2023

Mutation screening of SPTLC1 and SPTLC2 in amyotrophic lateral sclerosis.

Human genomics
2023

Clinical feature difference between juvenile amyotrophic lateral sclerosis with SPTLC1 and FUS mutations.

Chinese medical journal
2023

Early onset hereditary neuronopathies: an update on non-5q motor neuron diseases.

Brain : a journal of neurology
2022

A de novo c.113 T > C: p.L38R mutation of SPTLC1: case report of a girl with sporadic juvenile amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2022

An Atypical Presentation of Upper Motor Neuron Predominant Juvenile Amyotrophic Lateral Sclerosis Associated with TARDBP Gene: A Case Report and Review of the Literature.

Genes
2022

The VINE complex is an endosomal VPS9-domain GEF and SNX-BAR coat.

eLife
2023

A novel mutation in the ALS2 gene in an iranian kurdish family with juvenile amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2022

FUS-P525L Juvenile Amyotrophic Lateral Sclerosis and Intellectual Disability: Evidence for Association and Oligogenic Inheritance.

Neurology. Genetics
2022

Proximity-based labeling reveals DNA damage-induced phosphorylation of fused in sarcoma (FUS) causes distinct changes in the FUS protein interactome.

The Journal of biological chemistry
2022

ALS2-Related Motor Neuron Diseases: From Symptoms to Molecules.

Biology
2021

Juvenile Amyotrophic Lateral Sclerosis: A Review.

Genes
2022

Juvenile amyotrophic lateral sclerosis associated with biallelic c.757delG mutation of sorbitol dehydrogenase gene.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2022

The expanding clinical and genetic spectrum of alsin-related disorders: the first cohort of Brazilian patients.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2021

Generation and characterization of an endogenously tagged SPG11-human iPSC line by CRISPR/Cas9 mediated knock-in.

Stem cell research
2021

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis.

JAMA neurology
2021

A rare case of juvenile amyotrophic lateral sclerosis.

The Turkish journal of pediatrics
2022

A case of juvenile-onset amyotrophic lateral sclerosis with a de novo frameshift FUS gene mutation presenting with bilateral abducens palsy.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2021

The heterozygous deletion c.1509_1510delAG in exon 14 of FUS causes an aggressive childhood-onset ALS with cognitive impairment.

Neurobiology of aging
2021

Genotype-phenotype correlation in seven motor neuron disease families with novel ALS2 mutations.

American journal of medical genetics. Part A
2020

Uniparental Disomy Leading to a Rare Juvenile Form of ALS.

Journal of pediatrics, perinatology and child health
2020

A Human iPSC Line Carrying a de novo Pathogenic FUS Mutation Identified in a Patient With Juvenile ALS Differentiated Into Motor Neurons With Pathological Characteristics.

Frontiers in cellular neuroscience
2021

FUS mutation is probably the most common pathogenic gene for JALS, especially sporadic JALS.

Revue neurologique
2020

The ALS-related σ1R E102Q Mutant Eludes Ligand Control and Exhibits Anomalous Response to Calcium.

International journal of molecular sciences
2021

Juvenile amyotrophic lateral sclerosis with complex phenotypes associated with novel SYNE1 mutations.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2020

A de novo c.1509dupA:p.R503fs mutation of FUS: report of a girl with sporadic juvenile amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2020

Description of combined ARHSP/JALS phenotype in some patients with SPG11 mutations.

Molecular genetics &amp; genomic medicine
2020

Distinct Regulation of σ 1 Receptor Multimerization by Its Agonists and Antagonists in Transfected Cells and Rat Liver Membranes.

The Journal of pharmacology and experimental therapeutics
2020

A juvenile ALS-like phenotype dramatically improved after high-dose riboflavin treatment.

Annals of clinical and translational neurology
2020

Chinese families with autosomal recessive hereditary spastic paraplegia caused by mutations in SPG11.

BMC neurology
2019

A juvenile sporadic amyotrophic lateral sclerosis case with P525L mutation in the FUS gene: A rare co-occurrence of autism spectrum disorder and tremor.

Journal of the neurological sciences
2018

Clinical and Genetic Features of Patients with Juvenile Amyotrophic Lateral Sclerosis with Fused in Sarcoma (FUS) Mutation.

Medical science monitor : international medical journal of experimental and clinical research
2018

Clinical presentation and natural history of infantile-onset ascending spastic paralysis from three families with an ALS2 founder variant.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2018

SIGMAR1 gene mutation causing Distal Hereditary Motor Neuropathy in a Portuguese family.

Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
2018

A novel SETX gene mutation associated with Juvenile amyotrophic lateral sclerosis.

Brain and behavior
2017

The investigation of genetic and clinical features in Chinese patients with juvenile amyotrophic lateral sclerosis.

Clinical genetics
2017

A novel mutation of BICD2 gene associated with juvenile amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2016

A Novel Missense Mutation of the DDHD1 Gene Associated with Juvenile Amyotrophic Lateral Sclerosis.

Frontiers in aging neuroscience
2016

Mitochondria-associated membrane collapse is a common pathomechanism in SIGMAR1- and SOD1-linked ALS.

EMBO molecular medicine
2016

Mitochondrial Membrane Protein-Associated Neurodegeneration Mimicking Juvenile Amyotrophic Lateral Sclerosis.

Pediatric neurology
2016

Juvenile amyotrophic lateral sclerosis: Classical wine glass sign on magnetic resonance imaging.

Journal of pediatric neurosciences
2016

De novo FUS P525L mutation in Juvenile amyotrophic lateral sclerosis with dysphonia and diplopia.

Neurology. Genetics
2015

Turkish families with juvenile motor neuron disease broaden the phenotypic spectrum of SPG11.

Neurology. Genetics
2016

CLEC4C p.K210del variant causes impaired cell surface transport in plasmacytoid dendritic cells of amyotrophic lateral sclerosis.

Oncotarget
2016

Utility of whole exome sequencing in evaluation of juvenile motor neuron disease.

Muscle &amp; nerve
2016

Identification of two novel ALS2 mutations in infantile-onset ascending hereditary spastic paraplegia.

Amyotrophic lateral sclerosis &amp; frontotemporal degeneration
2016

ALS5/SPG11/KIAA1840 mutations cause autosomal recessive axonal Charcot-Marie-Tooth disease.

Brain : a journal of neurology
Ver todos os 72 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Esclerose lateral amiotrófica juvenil

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. An Unusual Presentation of Juvenile Amyotrophic Lateral Sclerosis with Superoxide Dismutase 1 Mutation: Subacute Bulbar Palsy With Asymmetric Limb Weakness.
    Annals of Indian Academy of Neurology· 2026· PMID 41820266mais citado
  2. SIGMAR1 gene-related neuromuscular disorders - what do we know?
    Neurologia i neurochirurgia polska· 2026· PMID 41334667mais citado
  3. A novel heterozygous variant of FUS gene associated with juvenile ALS and premature tremor onset: a case report.
    Amyotrophic lateral sclerosis &amp; frontotemporal degeneration· 2026· PMID 40544342mais citado
  4. SYNE1 Deficiency Manifesting Primarily With Motor Neuron Disease.
    Neurology. Genetics· 2025· PMID 41322345mais citado
  5. Pathology of three ALS patients with FUS variants, including one likely benign Q23L variant lacking FUS inclusions.
    Human molecular genetics· 2025· PMID 40659544mais citado
  6. Gne deletion in adult mice can cause thrombocytopenia, anemia, myopathy, bleeding, and death.
    J Neuromuscul Dis· 2025· PMID 41417753recente
  7. Acquired hemophilia a in a female with minimal change disease and hypothyroidism: a rare case report.
    Ann Med Surg (Lond)· 2025· PMID 40901171recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:300605(Orphanet)
  2. MONDO:0017593(MONDO)
  3. Esclerose Lateral Amiotrofica(PCDT · Ministério da Saúde)
  4. GARD:11901(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Q3475952(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Esclerose lateral amiotrófica juvenil
Compêndio · Raras BR

Esclerose lateral amiotrófica juvenil

ORPHA:300605 · MONDO:0017593
🇧🇷 Brasil SUS
CEAF
1ARiluzolEdaravona
Geral
Prevalência
<1 / 1 000 000
Herança
Autosomal recessive
CID-10
G12.2 · Doença do neurônio motor
Início
Childhood
Prevalência
0.0 (Europe)
MedGen
UMLS
C3468114
EuropePMC
Wikidata
Papers 10a
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