Speech and Language Delays Associated With New-Onset Seizures Revealing Dandy-Walker Variant

Area of Science:

• Pediatric Neurology
• Developmental Neuroscience
• Congenital Malformations

Background:

• Dandy-Walker syndrome is a rare congenital anomaly characterized by cerebellar vermis hypoplasia, enlarged fourth ventricle, and posterior fossa dilation.
• It is the most common posterior fossa malformation, typically diagnosed in infants under one year old.

Observation:

• A seven-year-old boy presented with neonatal hypotonia, delayed walking, and speech/language difficulties.
• Brain MRI revealed isolated inferior vermian hypoplasia, consistent with an uncommon Dandy-Walker variant, with no other CNS or systemic anomalies.
• The patient developed new-onset, recurrent unprovoked seizures.

Findings:

• The case presents an isolated inferior vermian hypoplasia, an atypical presentation of Dandy-Walker syndrome.
• The occurrence of new-onset seizures in the context of a less severe posterior fossa anomaly poses diagnostic and therapeutic challenges.

Implications:

• This case underscores the importance of pediatrician-psychiatrist collaboration in managing developmental delay.
• It highlights the need for careful evaluation and management of seizures in patients with posterior fossa anomalies, even atypical variants.
• Further research may elucidate the relationship between isolated vermian hypoplasia and seizure disorders.