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Dementia paralytica in a fifteen-year-old boy
J Goeman1, I Hoksbergen, B A Pickut
1Department of Neurology, AZ Middelheim, Antwerpen, Belgium.
Insights
Congenital syphilis can cause severe cognitive deterioration and neurological issues in children, leading to dementia paralytica. Early syphilis screening is crucial for all patients with cognitive decline.
Area of Science:
- Neurology
- Infectious Diseases
- Pediatrics
Background:
- Congenital syphilis is a serious infection transmitted from mother to child.
- Neurosyphilis can manifest with diverse and severe neurological symptoms.
Observation:
- A 15-year-old boy presented with progressive cognitive decline, ataxia, and pupillary abnormalities since age 7.
- Positive syphilis serology (VDRL 1:256, TPHA 1:163840) and cerebrospinal fluid findings (VDRL 1:16) confirmed neurosyphilis.
Findings:
- The patient was diagnosed with congenitally acquired dementia paralytica, linked to latent syphilis in his parents.
- Penicillin treatment led to partial neurological recovery, despite persistent cognitive impairment.
Implications:
- This case highlights the importance of considering congenital syphilis in the differential diagnosis of pediatric cognitive deterioration.
- Routine syphilis screening in patients with unexplained neurological and cognitive decline is recommended, irrespective of age.
Abstract:
A 15-year-old boy, whose history revealed an unremarkable pregnancy, birth and neonatal period and who had shown a normal motor and mental development, presented at the hospital with deterioration of cognitive functions since the age of 7. He was bedridden with manifest ataxia involving all limbs, anisocoria and a sluggish to absent pupil reaction to light. Syphilis serology was positive with a Venereal Disease Research Laboratory (VDRL) titer of 1:256 and a Treponema pallidum Haemagglutination Assay (TPHA) titer of 1:163840. Cerebrospinal fluid (CSF) protein concentration was 55 mg/dl and CSF-leucocyte count was 14/mm3 (85% mononuclear cells). CSF-VDRL-titer was 1:16. A diagnosis of congenitally acquired dementia paralytica was made, since the boy's parents' clinical exam and serology results were suggestive for latent syphilis. Although cognition was still very much deteriorated five months following penicillin treatment, clinical examination revealed partial recuperation. Screening for syphilis should be part of routine testing in every subject presenting with cognitive deterioration, regardless of age.