Secondary neurodevelopmental syndrome

Secondary Neurodevelopmental Syndromes involves significant neurodevelopmental features that do not fulfil the diagnostic requirements of any of the specific Neurodevelopmental Disorders that are judged to be a direct pathophysiological consequence of a medical condition not classified under mental and behavioural disorders, based on evidence from the history, physical examination, or laboratory findings.

6E60.0 Secondary Speech or Language Syndrome involves significant difficulties in the acquisition and execution of specific speech or language functions (e.g., errors of pronunciation, articulation, or phonology) that arise during the developmental period and persist substantially beyond the expected age.

6E60.Y Other Specified Secondary Neurodevelopmental Syndrome should be used when the syndrome is characterized by the presence of significant difficulties in the acquisition and execution of specific intellectual, motor coordination, or social functions that persist substantially beyond the expected age.

For presentations characterized by significant difficulties arising during the developmental period in the acquisition and execution of specific intellectual, motor coordination, or social functions that do not fulfil the diagnostic requirements of Disorders of Intellectual Development, Autism Spectrum Disorder or Stereotyped Movement Disorder, the following diagnosis may be appropriate:

6E60.Y Other Specified Secondary Neurodevelopmental Syndrome

Essential (Required) Features:

Note: Presentations that meet the diagnostic requirements of Disorders of Intellectual Development, Autism Spectrum Disorder, or Stereotyped Movement Disorder and are judged to be the direct pathophysiological consequence of a medical condition are not diagnosed as Secondary Neurodevelopmental Syndrome because, by convention, these conditions are diagnosed regardless of whether or not they are caused by a medical condition classified elsewhere.

• The presence of significant difficulties arising during the developmental period in the acquisition and execution of specific intellectual, motor coordination, or social functions that do not fulfil the diagnostic requirements of Disorders of Intellectual Development, Autism Spectrum Disorder, or Stereotyped Movement Disorder and persist substantially beyond the expected age.
• The symptoms are judged to be the direct pathophysiological consequence of a medical condition with onset during the developmental period, based on evidence from history, physical examination, or laboratory findings. This judgment depends on establishing that:

• The medical condition is known to be capable of producing the symptoms;
• The course of developmental difficulties (e.g., onset, remission, response of the neurodevelopmental symptoms to treatment of the etiological medical condition) is consistent with causation by the medical condition; and
• The symptoms are not better accounted for by a Neurodevelopmental Disorder (e.g., a Disorders of Intellectual Development, Autism Spectrum Disorder, Developmental Motor Coordination Disorder) or the effects of a medication or substance.
• The symptoms are a specific focus of clinical attention.

Boundaries with Other Disorders and Conditions (Differential Diagnosis):

• Boundary with Dementia with onset during the developmental period: Difficulties in the acquisition or execution of specific intellectual or social functions with onset during the developmental period (i.e., prior to age 18) that represent a decline from a previous level of functioning could be diagnosed as Dementia if all diagnostic requirements for Dementia are met and the impairments are known to be caused by an etiology that is specifically associated with Dementia. Otherwise, if the impairments are known to be due to a medical condition and diagnostic requirements for another Neurodevelopmental Disorder (e.g., Intellectual Developmental Disorder) are not met, a diagnosis of Other Specified Secondary Neurodevelopmental Disorder should be considered.
• Boundary with Disorders of Intellectual Development or Autism Spectrum Disorder: If the symptoms meet the diagnostic requirements of Disorders of Intellectual Development or Autism Spectrum Disorder and are judged to be the direct pathophysiological consequence of a medical condition with onset during the prenatal or developmental period (e.g., Fragile X Syndrome), both Disorder of Intellectual Development or Autism Spectrum Disorder and the underlying medical condition should be diagnosed, and a diagnosis of Other Specified Secondary Neurodevelopmental Syndrome is not assigned. However, if the diagnostic requirements of a Disorder of Intellectual Development or Autism Spectrum Disorder are not fully met (e.g., limitations in intellectual functioning without limitations in adaptive functioning) and the symptoms are attributed to a medical condition with onset during the prenatal or developmental period, a diagnosis of Other Specified Secondary Neurodevelopmental Syndrome may be assigned.
• Boundary with Developmental Motor Coordination Disorder: In Developmental Motor Coordination Disorder, individuals exhibit significant delays in the acquisition of gross and fine motor skills during the developmental period and impairment in the execution of coordinated motor skills that manifest in clumsiness, slowness, or inaccuracy of motor performance. If the difficulties with motor coordination are solely attributable to a Disease of Nervous System (e.g., cerebral palsy, muscular dystrophy), a Disease of the Musculoskeletal System or Connective Tissue, a sensory impairment (especially severe visual impairment), or joint hypermobility, a diagnosis of Other Specified Secondary Neurodevelopmental Syndrome should be assigned rather than Developmental Motor Coordination Disorder.
• Boundary with Stereotyped Movement Disorder: Stereotyped Movement Disorder is a Neurodevelopmental Disorder that is characterized by the presence of persistent voluntary, repetitive, stereotyped movements (e.g., body rocking, head banging) that result in significant interference with the ability to engage in normal daily activities or result in severe bodily injury. Stereotyped Movement Disorder is diagnosed even if it is judged to be caused by a medical condition classified elsewhere, and a diagnosis of Other Specified Secondary Neurodevelopmental Syndrome is not assigned.
• Boundary with other Neurodevelopmental Disorders: The diagnosis of Secondary Neurodevelopmental Disorder should be assigned instead of other Neurodevelopmental Disorders when the symptoms are judged to be due to an underlying medical condition. (This does not apply to Disorders of Intellectual Development, Autism Spectrum Disorder, or Stereotyped Movement Disorder.)
• Boundary with developmental difficulties caused by substances or medications, including withdrawal effects: When establishing a diagnosis of Other Specified Secondary Neurodevelopmental Syndrome, it is important to rule out the possibility that a medication or substance is causing difficulties in the acquisition or execution of specific intellectual, motor, or social functions instead of or in addition to the etiological medical condition. This involves first considering whether any of the medications being used to treat the medical condition are known to cause developmental difficulties at the dose and duration at which it has been administered. Second, a temporal relationship between the medication use and the onset of the developmental difficulties should be established (i.e., the developmental difficulties began after administration of the medication).

Potentially Explanatory Medical Conditions (examples):

Brain disorders and general medical conditions that have been shown to be capable of producing long-lasting intellectual impairment, poor social functioning, learning difficulties and disruptions in attentional processes include:

• Diseases of the Nervous System (e.g., acquired epileptic aphasia [Landau Kleffner Syndrome], autoimmune encephalitis, encephalopathy)
• Developmental Anomalies (e.g., Rett Syndrome)
• Diseases of the Visual System (e.g., congenital blindness, vision impairment)
• Endocrine, Nutritional or Metabolic Diseases (e.g., diabetes mellitus, hyper- or hypothyroidism, Lesch-Nyhan Syndrome, Lysosomal Diseases such as neuronal ceroid, lipofuscinosis or sphingolipidosis, mucolipidosis, phenylketonuria)
• Injury, Poisoning or Certain Other Consequences of External Causes (e.g., brain injury, concussion, traumatic haemorrhage)
• Neoplasms (e.g., neoplasms of brain or meninges)

Brain disorders and general medical conditions that have been shown to be capable of producing long-lasting movement dysfunction or motor impairment include:

• Diseases of the Nervous System (e.g., cerebral palsy, Huntington disease, muscular dystrophy, Parkinson disease, tardive dyskinesia)
• Developmental Anomalies (e.g., Ehlers-Danlos Syndrome, Rett Syndrome)
• Endocrine, Nutritional or Metabolic Diseases (e.g., Lesch-Nyhan Syndrome)