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Paeds Casesneurology-neurodisability-and-neuromuscular

Paeds Cases · neurology-neurodisability-and-neuromuscular

Secondary headache and raised intracranial pressure — structured clinical encounter

Structured encounter testing the assessment of a fourteen-year-old girl who presents with obesity, a three-month history of daily headache, transient visual obscurations, pulsatile tinnitus, and new double vision, found on fundoscopy to have bilateral papilloedema: the recognition of the red-flag pattern, the Friedman 2013 diagnostic work-up with magnetic resonance imaging and venography and a lumbar puncture opening pressure, the separation of idiopathic from secondary intracranial hypertension, and the stepwise management that protects vision with weight advice, acetazolamide, and serial visual surveillance.

structured clinical encounter
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Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A fourteen-year-old girl is referred to your general paediatric clinic with a three-month history of daily headache. The headache is present on waking, is described as a pressure across her forehead, worsens when she bends or strains, and is accompanied by early-morning nausea. Over the last month she has had episodes of her vision greying out when she stands up quickly, a whooshing sound in her ears in time with her pulse, and new horizontal double vision. Her body mass index is well above the ninety-fifth centile, she takes minocycline for acne, and her mother has type two diabetes. On examination her blood pressure is normal, her neurological examination is normal apart from a bilateral sixth-nerve palsy, and fundoscopy shows bilateral optic-disc swelling with blurred margins.

Task 1 — Recognise the red-flag pattern and form a problem representation (3 minutes)

From the history and the examination, identify the features that mark this as a secondary headache rather than a primary one, and explain why papilloedema is the pivot of the assessment. State your problem representation in one sentence, and explain the significance of the minocycline and the obesity for the differential diagnosis. [8] [3]

Task 2 — Reproduce the diagnostic criteria and plan the work-up (4 minutes)

State the Friedman 2013 revised diagnostic criteria for the pseudotumour cerebri syndrome, naming the specific imaging required to exclude a secondary cause and the role of the lumbar puncture. Explain why the lumbar puncture is performed only after imaging, and describe the cerebrospinal-fluid opening-pressure threshold and its interpretation in a child, including the caveat around the paediatric value. [5] [6]

Task 3 — Separate idiopathic from secondary intracranial hypertension (3 minutes)

The magnetic resonance imaging with venography is normal and the lumbar puncture opening pressure is 330 millimetres with normal cerebrospinal-fluid composition. Explain how you decide whether this is idiopathic or secondary intracranial hypertension in light of the minocycline, and state the first management step that applies in either case. [5] [7]

Task 4 — The management plan and the conversation about vision (5 minutes)

Outline the stepwise definitive management, including the weight advice, the acetazolamide and its dose, the monitoring of serum bicarbonate and potassium, and the surgical options for threatened vision. Explain why vision is the governing outcome and describe the serial surveillance that defines the follow-up. Finally, outline the conversation you would have with the girl and her mother about prognosis, adherence, and the circumstance that would make this a neuro-ophthalmological emergency. [7] [9] [8]

References

  1. [1]Lewis DW; Ashwal S; Dahl G; et al Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society Neurology, 2002.PMID 12196640
  2. [2]Langdon R; DiSabella MT Pediatric Headache: An Overview Curr Probl Pediatr Adolesc Health Care, 2017.PMID 28366491
  3. [3]Yonker M Secondary Headaches in Children and Adolescents: What Not to Miss Curr Neurol Neurosci Rep, 2018.PMID 30058035
  4. [4]Park EG; Yoo IH The diagnostic values of red flags in pediatric patients with headache Brain Dev, 2022.PMID 35568652
  5. [5]Friedman DI; Liu GT; Digre KB Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children Neurology, 2013.PMID 23966248
  6. [6]Inger HE; Rogers DL; McGregor ML; et al Diagnostic criteria in pediatric intracranial hypertension J AAPOS, 2017.PMID 29081363
  7. [7]Raoof N; Hoffmann J Diagnosis and treatment of idiopathic intracranial hypertension Cephalalgia, 2021.PMID 33631966
  8. [8]Gaier ED; Heidary G Pediatric Idiopathic Intracranial Hypertension Semin Neurol, 2019.PMID 31847041
  9. [9]Bulkowstein Y; Nitzan-Luques A; Schnapp A; et al The manifestations of metabolic acidosis during acetazolamide treatment in a cohort of pediatric idiopathic intracranial hypertension Pediatr Nephrol, 2024.PMID 37480382
  10. [10]Wilne S; Collier J; Kennedy C; et al Presentation of childhood CNS tumours: a systematic review and meta-analysis Lancet Oncol, 2007.PMID 17644483
  11. [11]Wilne S; Koller K; Collier J; et al The diagnosis of brain tumours in children: a guideline to assist healthcare professionals in the assessment of children who may have a brain tumour Arch Dis Child, 2010.PMID 20371594
  12. [12]Sheridan DC; Waites B; Lezak B; et al Clinical Factors Associated With Pediatric Brain Neoplasms Versus Primary Headache: A Case-Control Analysis Pediatr Emerg Care, 2020.PMID 29135901