Course Content
Neuroscience-III Module — Final Year MBBS

FINAL YEAR MBBS | KMU CURRICULUM

AIM Student Memory Support

Topic 10: Acquired & Hereditary Neuropathies

Neuroscience Module | Medicine & Pediatrics

High-Yield Revision • Clinical Associations • KMU Examination Focus

1. High-Yield Flashcards

Tap each question to reveal the answer.

What is the difference between mononeuropathy and polyneuropathy?
Mononeuropathy affects one peripheral nerve; polyneuropathy affects multiple nerves, usually symmetrically and distally.
Why does length-dependent polyneuropathy begin in the feet?
The longest peripheral axons are affected first, causing distal sensory loss that progresses proximally.
Which sensory abnormalities distinguish small-fibre from large-fibre neuropathy?
Small-fibre: burning pain and impaired temperature sensation. Large-fibre: impaired vibration and joint position sense with sensory ataxia.
What nerve conduction abnormality suggests axonal neuropathy?
Reduced sensory or motor response amplitudes, reflecting loss of functioning axons.
Which nerve conduction findings suggest demyelinating neuropathy?
Marked conduction slowing, prolonged distal latencies, conduction block or temporal dispersion.
Which clinical pattern suggests diabetic distal symmetric polyneuropathy?
Gradual bilateral burning or numbness of feet, reduced ankle reflexes and loss of protective sensation.
What is the characteristic presentation of Guillain–Barré syndrome?
Acute, usually symmetrical progressive limb weakness with diminished or absent tendon reflexes.
What CSF finding supports Guillain–Barré syndrome?
Albuminocytologic dissociation: elevated CSF protein with relatively few cells. CSF may be normal early.
Which disease-modifying therapies are used in Guillain–Barré syndrome?
Intravenous immunoglobulin or plasma exchange when indicated. Corticosteroids alone are ineffective in typical GBS.
What clinical time course helps distinguish CIDP from typical GBS?
CIDP progresses or relapses over more than eight weeks; typical GBS reaches its nadir within four weeks.
Which childhood findings suggest Charcot–Marie–Tooth disease?
Slowly progressive distal weakness, foot drop, pes cavus, muscle wasting and reduced ankle reflexes.
What is the main pathological distinction between CMT1 and CMT2?
CMT1 is predominantly demyelinating; CMT2 is predominantly axonal.
Which genetic abnormality is a classic cause of CMT1A?
Duplication of the PMP22 gene.
What is hereditary neuropathy with liability to pressure palsies (HNPP)?
An inherited neuropathy causing recurrent focal nerve palsies after minor compression.
What is the primary goal of managing common hereditary neuropathies?
Preserve mobility and independence through rehabilitation, orthoses, deformity management and family-centred care.

2. Mnemonics

Mnemonic 1 — Important Acquired Causes

Mnemonic Word: DINT

Meaning:

  • D — Diabetes and other metabolic diseases
  • I — Inflammatory and infective causes
  • N — Nutritional deficiencies
  • T — Toxins and drugs

A compact recall aid for major systemic acquired causes. Focal nerve entrapment is another important category.

Mnemonic 2 — Peripheral Neuropathy Assessment

Mnemonic Word: DART

Meaning:

  • D — Distribution: focal, multifocal or symmetric
  • A — Axonal or demyelinating mechanism
  • R — Reflex and sensory-motor findings
  • T — Time course: acute, subacute or chronic

Mnemonic 3 — Childhood CMT Recognition

Mnemonic Word: FEET

Meaning:

  • F — Foot drop and frequent falls
  • E — Elevated foot arch (pes cavus)
  • E — Extensor weakness and distal wasting
  • T — Tendon reflexes reduced, especially ankle jerks

3. Memory Tables

Axonal versus Demyelinating Neuropathy

Feature Axonal Demyelinating
Primary injury Nerve axon Myelin sheath
Nerve conduction Reduced response amplitude Marked slowing; possible conduction block
Typical examples Many diabetic neuropathies; CMT2 CIDP; CMT1
Diagnostic implication Loss of functioning fibres Impaired electrical conduction

Both axonal and demyelinating mechanisms can occur in acquired or hereditary neuropathies.

4. Rapid Revision Points — Last-Minute Revision

Must Remember:

  • Stocking–glove sensory loss indicates length-dependent polyneuropathy.
  • Mononeuritis multiplex produces painful, asymmetric involvement of multiple peripheral nerves.
  • Small-fibre neuropathy may have normal routine nerve conduction studies.
  • Reduced ankle reflexes support peripheral nerve dysfunction.
  • Diabetes is an important cause of acquired distal symmetric neuropathy.
  • GBS requires urgent monitoring for respiratory failure and autonomic dysfunction.
  • CIDP may respond to corticosteroids, IVIG or plasma exchange.
  • CMT usually causes slowly progressive distal weakness with foot deformity.
  • Nerve conduction studies classify the physiological pattern; genetic testing may confirm inherited disease.
  • Neuropathic pain management, foot protection, rehabilitation and orthoses reduce complications and disability.

5. Clinical Memory Hooks

Clinical Hook 1 — Diabetic Neuropathy

Long-standing diabetes with bilateral burning feet and absent ankle reflexes → distal symmetric polyneuropathy.

Clinical Hook 2 — Acute Immune Neuropathy

Ascending weakness after infection with areflexia → suspect GBS and assess respiratory function urgently.

Clinical Hook 3 — Hereditary Neuropathy

Childhood foot drop, pes cavus, distal wasting and affected relatives → suspect Charcot–Marie–Tooth disease.

Clinical Hook 4 — Vasculitic Neuropathy

Painful, stepwise deficits affecting separate nerve territories → mononeuritis multiplex; consider vasculitis.

6. Starred High-Yield Exam Points

Priority KMU Concepts

⭐ 1. Reduced nerve conduction amplitudes favour axonal loss; marked conduction slowing favours demyelination.

⭐ 2. Rapid progressive weakness with areflexia suggests GBS; respiratory and autonomic deterioration are major dangers.

⭐ 3. Albuminocytologic dissociation supports GBS, but normal early CSF does not exclude it.

⭐ 4. IVIG or plasma exchange treats typical GBS when indicated; corticosteroids alone are ineffective.

⭐ 5. CMT1 is predominantly demyelinating; CMT2 is predominantly axonal. PMP22 duplication classically causes CMT1A.

⭐ 6. A positive family history supports hereditary neuropathy, but its absence does not exclude an inherited cause.

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