🧠 MOCK OSCE · FCPS, MCPS, MD PAEDIATRICS ⏱ 10 min · SPASTIC PARAPLEGIA

Spastic Paraplegia · Short Case

Candidate task: perform focused neurological examination on a child with spastic paraplegia.
Then discuss differential diagnosis, investigations, management & follow‑up.
Pre‑exam Protocol
⏤ · Wash, Warm, Introduce, Position, Expose, Approach ▾

Standard pre‑examination protocol – must be demonstrated:

🖐 Wash hands with sterilizing solution.
🔥 Warm hands and stethoscope.
👋 Introduce yourself to child & parent.
🧍 Position child: supine, then standing for gait.
👕 Exposure — allow full neurological exam.
➡️ Approach from the right side.
✔ CPSP marker: Pre‑exam Protocol is observed and scored.
1. Clinical Examination (≈6 min)
02 General Look (Inspection from end of bed) ▾

Key observations:

  • Gait: stiff, scissoring, toe‑walking, circumduction.
  • Posture: lumbar lordosis, adducted hips, flexed knees.
  • Lower limbs: muscle wasting (disuse), foot deformities (pes cavus, equinovarus).
  • Upper limbs: usually normal (pure HSP).
  • Speech: dysarthria (if complicated HSP).
  • Eye signs: optic atrophy (complicated HSP).
  • Skin: neurocutaneous stigmata (if syndromic).
  • Back: scoliosis, midline defects.
👁 Red flags: rapid progression, upper limb involvement, bulbar signs, cognitive decline → complicated HSP / other diagnosis.
03 Focused Neurological Examination ▾

Systematic motor & sensory exam:

  • Gait: observe walking, heel‑toe, toe‑walking, scissoring, circumduction.
  • Lower limbs: tone – spasticity (velocity‑dependent), clasp‑knife phenomenon.
  • Power: weakness (hip flexors, knee extensors, ankle dorsiflexors).
  • Reflexes: brisk knee/ankle jerks, crossed adductor reflex, clonus (sustained).
  • Plantars: extensor (Babinski positive).
  • Sensation: usually normal (pure HSP); may be impaired (complicated HSP).
  • Upper limbs: usually normal (pure HSP).
  • Cranial nerves: optic atrophy, dysarthria (complicated HSP).
  • Coordination: usually normal (pure HSP).
  • Back: inspect for scoliosis, midline defects.
🔍 Key: pure HSP = spastic paraparesis + urinary urgency + normal upper limbs + normal sensation.
04 General Physical Exam (Hands → Face → Chest → Abdomen → Limbs) ▾

Systematic examination:

  • Hands: pes cavus, hammer toes (if complicated HSP).
  • Face: dysarthria, optic atrophy (complicated HSP).
  • Eyes: fundoscopy (optic atrophy, retinitis pigmentosa).
  • Chest: scoliosis, respiratory effort.
  • Abdomen: bladder distension (neurogenic bladder).
  • Skin: neurocutaneous stigmata (if syndromic).
  • Back: scoliosis, midline defects (spina bifida occulta).
📏 Anthropometry: weight, height – plot on growth charts.
05 Developmental Assessment & Associated Signs ▾

Assess:

  • Motor milestones: delayed (walking).
  • Cognitive: intellectual disability (complicated HSP).
  • Speech: dysarthria (complicated HSP).
  • Behaviour: may be normal.
  • Bladder: urgency, frequency, incontinence.
🧠 Red flags: cognitive decline, dysarthria, upper limb involvement → complicated HSP.

📋 Case Presentation – (fill in during exam)

This is a _____-year-old _____ child, referred for _____ (stiff legs / difficulty walking / frequent falls). On examination, the child appears _____ (well/unwell), with _____ (scissoring gait / toe‑walking). Neurological exam: tone in lower limbs _____ (increased/spastic), power _____ (reduced), reflexes _____ (brisk), clonus _____ (present/absent), plantars _____ (extensor). Upper limbs: tone _____ (normal / increased), power _____, reflexes _____. Sensation: _____ (normal / impaired). Eye findings: fundi _____ (normal / optic atrophy). Growth: weight _____ percentile, height _____ percentile. Associated signs: _____ (pes cavus, scoliosis, urinary urgency).

2. Viva Discussion (≈4 min)
06 Viva · Differential, Investigations, Management, Follow‑up ▾
🔹 Differential Diagnosis

• Hereditary Spastic Paraplegia (HSP) – pure or complicated.
• Cerebral Palsy (spastic diplegia) – perinatal insult, static.
• Spinal cord compression – tumour, trauma, infection (TB).
• Adrenoleukodystrophy – progressive, adrenal insufficiency.
• Friedreich ataxia – ataxia, sensory loss, cardiomyopathy.
• Vitamin B12 deficiency – subacute combined degeneration.
• HTLV-1 associated myelopathy (HAM) – tropical spastic paraparesis.
• Syringomyelia – sensory dissociation, segmental muscle wasting.
• Multiple sclerosis – relapsing‑remitting, optic neuritis.
• Dopa‑responsive dystonia – diurnal variation, responds to L‑dopa.
• Primary lateral sclerosis – upper motor neuron only.
• Functional – non‑organic.

🔹 Investigations – Diagnosis

• Brain MRI – periventricular leukomalacia (CP), atrophy (HSP).
• Spinal MRI – cord compression, atrophy, syrinx.
• Genetic testing – SPG genes (SPG4, SPG3A, SPG11, etc.).
• NCS/EMG – to exclude peripheral neuropathy.

🔹 Investigations – Aetiology

• Genetic testing – targeted panel for HSP genes.
• MRI brain – thin corpus callosum (SPG11), white matter changes.
• VLCFA – adrenoleukodystrophy.
• Vitamin B12, folate – nutritional.
• HTLV-1 serology – if travel/endemic area.
• CSF – if infection/demyelination suspected.

🔹 Investigations – Exclude Others

• MRI spine – cord compression, syrinx.
• CSF – oligoclonal bands (MS), infection.
• Evoked potentials – VEPs (optic neuritis).
• Metabolic screen – amino acids, organic acids.
• Copper/caeruloplasmin – Wilson disease (if mixed).

🔹 Investigations – Rule Out Complications

• Urodynamics – neurogenic bladder.
• Orthopaedics – scoliosis, hip dislocation.
• Ophthalmology – optic atrophy (complicated HSP).
• Audiology – hearing impairment (complicated).
• Physiotherapy – functional assessment.

🔹 Management – Across Organ Systems

Neurological

Spasticity: physiotherapy, stretching, botulinum toxin, baclofen (oral/IT), tizanidine.

Orthopaedic

Contractures: tendon releases, osteotomies; scoliosis: bracing, surgery; pes cavus: orthotics.

Bladder

Neurogenic bladder: anticholinergics (oxybutynin), intermittent catheterisation, urology referral.

Rehabilitation

Physiotherapy, OT, gait aids (ankle‑foot orthoses, walking sticks).

Genetic Counselling

Recurrence risk (AD, AR, X‑linked), carrier testing, prenatal diagnosis.

Pain Management

Muscle spasms: benzodiazepines, baclofen, gabapentin.

Nutritional

Vitamin B12 replacement (if deficient), folate.

Psychosocial

Support groups, counselling, school support.

📈 Prognosis

  • HSP – pure: slowly progressive; near‑normal life expectancy.
  • HSP – complicated: variable; depends on associated features.
  • SPG4 (most common): onset 2nd‑4th decade; slow progression.
  • SPG3A (early‑onset): childhood onset; slow progression.
  • SPG11 (complicated): thin corpus callosum, cognitive decline.
  • Cerebral palsy: static; non‑progressive.
  • Spinal cord compression: depends on cause; treatable.

📋 Follow‑up Schedule

  • HSP: annual neurology review (monitor progression).
  • Physiotherapy: regular (spasticity management).
  • Bladder: urology review if symptoms.
  • Orthopaedics: as needed (contractures, scoliosis).
  • Genetic: counselling for family members.
  • CP: multidisciplinary (neurology, orthopaedics, rehabilitation).
  • Transition: plan for adult care.
💡 Examiner expectation: ability to differentiate pure vs complicated HSP, identify red flags (upper limb involvement, cognitive decline, dysarthria), and discuss genetic testing and multidisciplinary management. Know the differentials: CP (static), cord compression, adrenoleukodystrophy.
📚 Visit paeds.online for more CPSP resources
Mock OSCE · Spastic Paraplegia · Based on Wyne‑Harris, Nelson & Pediatric Clinical Advisor