Onset at Age 4 Years: Causes & Reasons

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Possible Causes for Onset at Age 4 Years

Neuronal Ceroid Lipofuscinosis Type 9

[…] between ages 2 and 4 years Epilepsy Cognitive decline Ataxia Myoclonus Extrapyramidal symptoms Pyramidal symptoms Blindness at age 4-6 years Death before or in the second [emedicine.staging.medscape.com]

In this family, onset of the disorder occurred at 4 to 5 years of age, with affected children becoming silent, taciturn, and irritable. [cags.org.ae]

Onset before 6 months and after 2 years of age is considered unusual for CLN1/PPT-1. [4] Characteristic visual failure commonly described in the literature was conspicuously [neurologyindia.com]

Familial Temporal Lobe Epilepsy Type 8

Subject III:2, who was treated at our institution, had pyknolepsy, onset at age 3 years, with 4-Hz generalized spike-wave and polyspike-wave activity on EEG. [ncbi.nlm.nih.gov]

Age at onset averaged 14 ± 1.95 years (SE). None of the families contained an individual with glioblastoma. [ncbi.nlm.nih.gov]

They contained 43 individuals with idiopathic epilepsy (4 primary generalized; 31 focal; 3 focal and primary generalized; and 5 unclassifiable). [ncbi.nlm.nih.gov]

Ring Chromosome 20 Syndrome

Methods: We describe three children with [r(20)] syndrome in whom the onset of epilepsy (age at onset range: 4 years and 6 months to 9 years and 4 months) determined a kind [moh-it.pure.elsevier.com]

METHODS: We describe three children with [r(20)] syndrome in whom the onset of epilepsy (age at onset range: 4 years and 6 months to 9 years and 4 months) determined a kind [ncbi.nlm.nih.gov]

Seizure onset was at age 4 years, when she developed clonic seizures in sleep, followed by different types of seizures in wakefulness, losing her ability to speak. [karger.com]

Familial Infantile Myoclonic Epilepsy

CLINICAL AND EEG MANIFESTATIONS The most common age of onset of BMEI is between 4 months and 3 years of age, but later onset up to 4 years 8 months has been reported. 4, 5 [mhmedical.com]

CLINICAL AND EEG MANIFESTATIONS The most common age of onset of BMEI is between 4 months and 3 years of age, but later onset up to 4 years 8 months has been reported.4,5 The [neurology.mhmedical.com]

Prevalence ~1% to 2% of epilepsies that start before the age of 3 years. Age at onset 6 months to 3 years but also earlier (4 months) or later (4 years). [epilepsy.com]

Neuronal Ceroid Lipofuscinosis Type 3

It is characterized by severe visual impairment with onset around age 4–8 years, with a developmental course that includes dementia, blindness, epilepsy, speech problems, [kurs.statped.no]

Batten disease is one of the most common of the NCLs with the classic juvenile onset of symptoms between ages 4 and 8 years. [ggc.org]

Santavuori) 9-19 months; Late-infantile (Batten-Bielschowsky) 2-4 years; Variant (Batten) 5-7 years; Juvenile (Spielmeyer-Vogt) 4 years-puberty. [pediatricneurologybriefs.com]

Myoclonic-Astatic Epilepsy

CLINICAL AND EEG MANIFESTATIONS The most common age of onset of BMEI is between 4 months and 3 years of age, but later onset up to 4 years 8 months has been reported. 4, 5 [mhmedical.com]

Clinical description In 94% of cases, onset occurs during the first 5 years of life, most commonly between the ages of 3-4 years. [orpha.net]

Clinical context This syndrome is characterized by seizures that have onset between 6 months and 6 years of age (peak 2 to 4 years). [epilepsydiagnosis.org]

Childhood Absence Epilepsy

The age of onset is between 4–10 years with peak age between 5–7 years. [en.wikipedia.org]

Early onset absence epilepsy (EOAE) starting before the age of 4 years constitutes a rare subgroup of the idiopathic generalized epilepsies (IGEs). [ncbi.nlm.nih.gov]

Abstract Childhood absence epilepsy (CAE) is defined by absence seizures in a normally developing child, with onset between 4 and 10 years of age.(1) Typical absence seizures [ncbi.nlm.nih.gov]

Focal Epilepsy - Speech Disorder - Mental Retardation

Clinical context This syndrome is characterized by onset of seizures between 2 and 12 years of age (peak 4-5 years), with epileptic encephalopathy with continuous spike-and-wave [epilepsydiagnosis.org]

Age at onset Seizures 2 months to 12 years; peak at 4 to 5 years. EEG continuous spikes and waves during slow-wave sleep (CSWS), usually 1 to 2 years from seizure onset. [epilepsy.com]

Discussion CSWS is a sort of special epilepsy syndrome in childhood, commonly encountered in boys, with onset age of 2 months to 12 years old and peak age of 4 ～ 8 years old [jitm.hk]

Neuronal Ceroid Lipofuscinosis Type 4B

Among these, the classic late infantile type is caused by mutations in the tripeptidyl peptidase 1 ( TPP1 ) gene and is characterized by age of onset between 2–4 years, seizures [atm.amegroups.com]

[…] of 2 years was seen in seven children, and all phasic cerebral activity was unrecordable at 3-4 years of age; the clinical onset with regression in skills began at 1-2 years [jnnp.bmj.com]

Classic CLN2 disease presents between ages 1 to 4 years. [omicsonline.org]

Acquired Epileptic Aphasia

Age at onset Seizures 2 months to 12 years; peak at 4 to 5 years. EEG continuous spikes and waves during slow-wave sleep (CSWS), usually 1 to 2 years from seizure onset. [epilepsy.com]

Case 1 The first patient was a male who presented with complex partial seizures at 4 years of age. [intechopen.com]

Alpers Syndrome

Typically, children develop normally over the first few months or years with the average age at onset of 2 - 4 years, but onset can be as late as the third decade. [grantome.com]

The most common age of onset is between 2-4 years (ranges from 3 months to 36 years). [orpha.net]

Alpers syndrome is thought to be a uniformly fatal disorder, but the rate of neurological degeneration, degree of liver involvement and age of death vary considerably. [grantome.com]

Absence Seizure

In children who have absences that have onset before 4 years of age, 10% have GLUT1 deficiency (a mutation in SLC2A1 ). [medlink.com]

In children who have absences that have onset before 4 years of age, 10% have GLUT1 deficiency (a mutation in SLC2A1). [medlink.com]

Onset of absence seizures under the age of 4 years is observed in a rare subset of CAE patients, a proportion of whom have encephalopathy due to GLUT1 deficiency (see this [orpha.net]

Late-Infantile Neuronal Ceroid Lipofuscinosis

Symptom onset most commonly occurs in children aged 2-4 years, whereby the patients' prior development is normal. [symptoma.com]

A type of neuronal ceroid lipofuscinosis with onset usually between ages two and four. [medical-dictionary.thefreedictionary.com]

GM2 Gangliosidosis

A 9-year-old boy showed a progressive generalized dystonia, with onset at the age of 4 years, combined with mental deterioration and behavioral disturbances. [ncbi.nlm.nih.gov]

Onset during infancy leads to rapid neurodegeneration and death before 4 years of age. [ncbi.nlm.nih.gov]

Familial Temporal Lobe Epilepsy Type 1

Subject III:2, who was treated at our institution, had pyknolepsy, onset at age 3 years, with 4-Hz generalized spike-wave and polyspike-wave activity on EEG. [ncbi.nlm.nih.gov]

Age at onset averaged 14 ± 1.95 years (SE). None of the families contained an individual with glioblastoma. [ncbi.nlm.nih.gov]

They contained 43 individuals with idiopathic epilepsy (4 primary generalized; 31 focal; 3 focal and primary generalized; and 5 unclassifiable). [ncbi.nlm.nih.gov]

Catecholaminergic Polymorphic Ventricular Tachycardia Type 5

CPVT type 4 has an aberrant CALM1 gene, and the median age of symptom onset is 4 years of age. [jaclinicalreports.springeropen.com]

The age of CPVT onset is usually before 10 years, although the much later onset has been reported [4]. [austinpublishinggroup.com]

An aberrant TECRL gene is present in CPVT type 3, and the median age of symptom onset is 10 years. [jaclinicalreports.springeropen.com]

Papillon Lefevre Disease

The onset of symptoms is between 1 and 4 years of age. [ijdr.in]

[…] its onset between the ages of 1 and 4 years. [5] The sharply demarcated erythematous keratotic plaques may occur focally, but usually involve the entire surface of the palms [jiaomr.in]

The palmoplantar keratoderma typically has its onset between the ages 1 and 4 years [5]. [escholarship.org]

Infantile Neuronal Ceroid Lipofuscinosis

[…] of onset 10-18 months), Late Infantile NCL (LINCL, age of onset around 3 years) and Juvenile NCL (JNCL, age of onset 4-7 years). [ncl-netz.de]

It is characterized by onset between 4-7 years of age and death in the third decade of life. [preventiongenetics.com]

Age at onset ranged between 4-10 years. Behavioral problems were the initial symptoms in patients 13 and 14, leading to dementia in 4-5 years. [scielo.br]

Exotropia

Onset of ET below 4 years of age was associated with a higher risk for consecutive XT when compared to onset of strabismus >4 years of age. [onlinelibrary.wiley.com]

The most common age of onset is between 2 and 5 years of age. During the early stages, the child has straight eyes looking at anything closer than 3 to 4 feet. [drmilesburke.com]

Of 12 patients with onset of strabismus between 4 and 10 years of age, only one patient did not have any binocular vision. [onlinelibrary.wiley.com]

Polyarticular Juvenile Idiopathic Arthritis

Oligo- or polyarticular arthritis, disease onset over 6 years of age, duration of disease under 4 years. [healio.com]

The mean known age of uveitis onset was 13 years; disease onset was 4 years earlier, on average, in girls than in boys. [uveitis.org]

[…] polyarticular JIA ≥5 joints are affected peak age: 1-4 years; 7-10 years mainly affects small and medium joints systemic onset JIA (Still's disease, not to be confused with [radiopaedia.org]

ROHHAD Syndrome

There are multiple clinical characteristics, which include: - onset between 2 and 4 years of age - hyperphagia and rapidly increasing obesity - endocrine disorders: hypernatremia [sites.uclouvain.be]

• The typical age of onset is between 2 and 4 years. 9. sudden rapid weight gain and concomitant growth failure (median age at onset: 3 years) Hypothalmic and autonomic [slideshare.net]

In detail, the following symptoms consecutively manifest in children aged 2-4 years: Hyperphagia leading to acute-onset, rapidly progressive obesity. [symptoma.com]

Neuronal Ceroid Lipofuscinosis

Abstract Juvenile neuronal ceroid lipofuscinosis (JNCL) is characterized by severe visual impairment with onset around age 4-8 years, and a developmental course that includes [ncbi.nlm.nih.gov]

Age of onset is typically around age 4-7 years. Mutations in CLN5 have since been described outside of the Finnish population. [ncbi.nlm.nih.gov]

A late onset form in a small proportion of patients starts at the age of 4 to 10 years, but also leads to severe neurological deterioration. [ncbi.nlm.nih.gov]

Neuronal Ceroid Lipofuscinosis Type 10

The 12 females ranged in age from 3 to 12 years (mean=6.6; median=6), whereas the males ranged in age from 1 to 4 years (mean=3; median=4). [scielo.br]

Age at onset ranged between 4-10 years. Behavioral problems were the initial symptoms in patients 13 and 14, leading to dementia in 4-5 years. [scielo.br]

The onset of this form occurs during 4-8 years of age and is characterized by vision loss and progression to total blindness within 2 years, epilepsy, and motor and cognitive [scielo.br]

Krabbe Disease

4 Adult onset Begins anytime after 8 years of age Since Krabbe disease affects the nerve cells, many of the symptoms it causes are neurological. [verywell.com]

Type 1: Infantile: begins at age 3 – 6 months Type 2: Late infantile: begins at age 6 months – 3 years Type 3: Juvenile: begins at age 3 – 8 years Type 4: Adult onset: begins [myvmc.com]

We report a sporadic case of late onset GLD. The illness started at the age of 3 years and 8 months with spastic gait. [ncbi.nlm.nih.gov]

Myotonia Congenita Type Thomsen

Becker disease is more common, is autosomal recessive, has a later onset from 4 to 12 years of age, and tends to be more severe than the autosomal dominant form. [merckmanuals.com]

There is progressive loss of alpha-motor neurons in the anterior horn of the spinal cord 5 Age of onset is usually between 7 and 15 years ML 3 Select Answer to see Preferred [orthobullets.com]

Thomsen disease is autosomal dominant and manifests from infancy to 2 to 3 years of age. [merckmanuals.com]

Myoclonic Epilepsy of Infancy

CLINICAL AND EEG MANIFESTATIONS The most common age of onset of BMEI is between 4 months and 3 years of age, but later onset up to 4 years 8 months has been reported. 4, 5 [mhmedical.com]

Prevalence ~1% to 2% of epilepsies that start before the age of 3 years. Age at onset 6 months to 3 years but also earlier (4 months) or later (4 years). [epilepsy.com]

Clinical context This syndrome is characterized by the onset of myoclonic seizures between the ages of 6 months and 2 years, in some cases earlier (4 months) or later (2-4 [epilepsydiagnosis.org]

Metachromatic Leukodystrophy

The infantile form is characterized by progressive paralysis and dementia usually beginning before age 4 years and resulting in death about 5 years after onset of symptoms [merckmanuals.com]

There are three types of MLD: infantile (onset occurs within the first four years of life), juvenile (onset occurs between ages 4 and 16), and adult (onset occurs after age [britannica.com]

Five patients with early onset form of MLD, age ranging from 6 months to 4 years, will be included in this protocol and will be followed during 24 months. [clinicaltrials.gov]

Psoriatic Juvenile Idiopathic Arthritis

Results Among 361 children enrolled as CARRA-JPsA, 72.02% had symptom onset at > 4 years of age, with a male predominance and high prevalence of enthesitis. [jrheum.org]

RESULTS: Among 361 children enrolled as CARRA-JPsA, 72.02% had symptom onset at > 4 years of age, with a male predominance and high prevalence of enthesitis. [ncbi.nlm.nih.gov]

Oligoarticular JIA is the most common type of JIA and has a peak onset between the ages of 2 and 4 years. [verywell.com]

Autosomal Recessive Spastic Paraplegia Type 64

The signs and symptoms of spastic paraplegia type 3A usually appear before the age of 10; the average age of onset is 4 years. [ghr.nlm.nih.gov]

The age onset of symptoms ranged from 18 months to 17 years (mean age of onset, 9 years). [ajnr.org]

SPG3A is the most frequent cause of hereditary spastic paraplegia with onset before age 10 years. Neurology. 2006 Jan 10;66(1):112-4. [ghr.nlm.nih.gov]

Severe Combined Immunodeficiency due to Adenosine Deaminase Deficiency

Ten to 15% of patients have a delayed clinical onset by age 6-24 months, and a smaller percentage have a partial form of ADA deficiency with later onset between ages 4 years [orpha.net]