Categories
Internal Medicine

Motor Neuron Disease (MND)

Motor neuron disease (MND) is a spectrum of neurodegenerative disorders that selectively affect motor neurons.

Motor neuron disease (MND) is a spectrum of neurodegenerative disorders that selectively affect motor neurons.


Classification

TypeUMN degenerationLMN degeneration
Amyotrophic lateral sclerosis (ALS)YesYes
Hereditary spastic paraplegia (HSP)YesNo
Primary lateral sclerosis (PLS)YesNo
Progressive muscular atrophy (PMA)NoYes
Progressive bulbar palsy (PBP)NoYes, bulbar region
Pseudobulbar palsyYes, bulbar regionNo

Etiology

Mixed UMN & LMN (ALS) syndrome

  • Compressive myeloradiculopathy
  • HIV infection

Pure LMN syndrome

  • Multifocal motor neuropathy + conduction block
  • Pure motor chronic inflammatory demyelinating polyneuropathy
  • Toxic neuropathies

  • Myasthenia gravis
The Calgary Guide | http://calgaryguide.ucalgary.ca/

Pure UMN syndrome

  • Cord compression
  • B12 deficiency
The Calgary Guide | http://calgaryguide.ucalgary.ca/

Non-neurological features:

  • Respiratory:
    • Progressive respiratory failure (M/C cause of death)
  • ENT:
    • Dysphagia
  • Orthopaedics/neurosurgery:
    • Foot drop or other symptoms suggestive of compressive radiculopathy
  • Geriatric case:
    • Difficulty walking

Diagnosis

Mixed UMN & LMN (ALS) syndrome:

  • Nerve conduction studies/electromyography

  • MRI (spinal cord & brain)

  • Routine blood tests, thyroid function, serum electrophoresis

  • HIV test (if appropriate risk factors)

Pure LMN syndrome:

  • Genetic tests (if slowly progressive, suggesting spinal muscular atrophy)

  • Nerve conduction studies/electromyography for conduction block.

  • Routine blood tests, thyroid function, serum electrophoresis

Pure UMN syndrome

  • MRI (spinal cord & brain as appropriate to the level of the neurological signs)

  • B12/folate

  • Central motor conduction time (using transcranial magnetic stimulation techniques, not routinely available)

  • Lumbar puncture


Management

Multidisciplinary approach

  • Patient and carer support groups
  • Palliative care
Occupational therapy can help to alleviate some of the stiffness and stress of a motor neuron disease. | Judith Marcin, M. (2018). Motor neuron disease: Types, symptoms, causes, and treatments. Medical News Today. Retrieved 16 July 2018, from https://www.medicalnewstoday.com/articles/164342.php

Nutrition:

  • Percutaneous endoscopic gastrostomy (PEG)
    • Endoscopic medical procedure in which a PEG tube is passed into a patient’s stomach through the abdominal wall to provide a means of feeding when oral intake is not adequate

Respiratory care:

  • Non-invasive ventilation (NIV)
    • Use of airway support administered through a face (nasal) mask instead of an endotracheal tube
    • NIV is not the same as continuous positive airway pressure (CPAP), which applies a single level of positive airway pressure throughout the whole respiratory cycle. CPAP does not deliver ventilation but is occasionally used in conditions also treated with NIV

Drug therapy:

  • Riluzole

Prognosis

Poor prognosis:

  • Later age of onset
  • Bulbar involvement
  • Short diagnostic interval
  • ALS/PMA variant

Leave a Reply