diease

Acute Flaccid Myelitis


OVERVIEW OF Acute Flaccid Myelitis :

It is used to describe the patient with suddent onset of flabby limb without the known cause and with the lesions in the gray matter of the spinal cord.it is most common among children.childrens feel difficulty in moving their faces and walking.


EPIDEMIOLOGY :

Epidemiology: Over 90-95% of the cases is seen in children.


COMMON CLINICAL SIGNS AND SYMPTOMS :

  • Weakness in upper and lower limbs.

  • Flabby face  affecting the muscles of the face leading one side of the face to fall lower on the other.

  • Pain in arm and leg

  • Inability to urinate.

  • Respiratory muscles are affected leading to breathing problems.

  • Slurred speech

  • Fine paraesthesia in the toes or fingers.


DIAGNOSTIC :

Physical examination and patient history.

Neurological examinations to see check the

mental status(asking general questions to asses the status)

coordination of the movements: your neurologist may ask you to walk in a straight line or closing your eyes and touching your nose with the index finger.

Reflexes: reflexes are tested by tapping your body in a certain way with the soft hammer to rule out the muscle tone.if relexes are normal the mucle movements will be normal on tapping with the hammer.

Sensation: Neurologists touch the legs, arms, and other areas with the instruments such as tunning folk or dull needle and ask you to feel the sensations such as heat, cold, pain.

Autonomic nervous system: this is the system which monitors our visceral organs.neurologists look for pupils in rresponse to the light,blood pressure,pulse rate and heart rate.

An MRI of the spinal cord and Brain: suggestive of lesions in the gray matter.

Testing with cerebrospinal fluid(CSF): used to  analyse cell count and glucose level, protein, antibody produced, viral structures.it shows pleocytosis(increase in white blood cells)

Electromyelograph: measures the electrical activity of muscles and nervesLumbar puncture (CSF): Guillain Barre syndrome, suspicion of viral myelitis

Biochemistry: Creatine Kinase, Potassium, Magnesium, Phosphate

Urine for porpho-bilinogens, toxins: arsenic, lead etc.,


TREATMENT AND PROGNOSIS :

There is no specific treatment for AFM.neurologists suggests physical or occupational therapy to aid in arm or leg weakness.

  • Guillain Barre syndrome: IVIG, 2 g/kg over 2–5 d

  • Transverse myelitis: IV methyl-prednisolone 10–30 mg/kg, daily (max-1 g) for 3–5 d

  • Compressive myelopathy: spinal immobilization, surgical intervention, steroids (acute traumatic myelopathy)

  • Dermatomyositis, Myasthenia Gravis: Immunomodulation

  • Hypokalaemia: Intravenous potassium correction

While there have not been systematic pathologic descriptions of enterovirus D68-associated myelitis in humans, older literature from the polio era identified anterior horn cell death with an associated inflammatory response. This inflammatory response was evident with the anterior horns of the spinal cord and, in some cases, within the surrounding white matter. Thus, it was possible for patients with virally mediated anterior horn cell death to experience simultaneous inflammation-mediated white matter damage 

Acute-phase immune modulating therapies : 

Approach to AFM – For patients with only flaccid weakness (ie, only lower motor neuron involvement) and T2-hyperintense lesions restricted to the gray matter on spinal cord magnetic resonance imaging (MRI), we generally treat during the acute phase with intravenous immunoglobulin (IVIG) for a total dose of 2 g/kg given over four to five days. For patients with mixed upper and lower motor neuron involvement and/or gray and white matter lesions on spinal cord MRI, we generally treat using high-dose glucocorticoids (eg, methylprednisolone 30 mg/kg per day, maximum daily dose 1000 mg, given for four to five days) combined with plasma exchange, typically five to seven exchanges given over 8 to 14 days. Our approach is based upon clinical experience, case studies and animal data, but is unproven. In both cases, rehabilitation therapy is essential.

Efficacy data – Immune modulators for AFM have shown no clear signal of effectiveness.Patients have been treated with glucocorticoids, intravenous immunoglobulin (IVIG), and plasma exchange (similar to treatment for transverse myelitis), without prospective studies to document outcomes. Empiric treatments have also included interferon, antivirals, and other immunomodulatory agents.

 Rehabilitation — Early and regular rehabilitation therapies are essential to maximal recovery. The techniques used include activity-based therapy, electrical stimulation, and functional electrical stimulation.

Nerve transfer — A novel approach to treating the lower motor neuron deficits has been the use of nerve transfer procedures. These surgeries involve the identification of "donor" nerves in proximity to a denervated muscle, followed by the surgical transfer of the healthy nerve to a recipient target. These procedures have produced positive results in a subset of patients with AFM. The optimal timing of the surgery has not been firmly established and not every patient is a candidate for these proceded.


PROGNOSIS :

  • The prognosis in children is good when compared to the  adults.

  • Mortality is approximately 3-4% and usually is secondary to autonomic dysfunction and respiratory failure.


PREVENTION :

Since we don’t know what triggers AFM in a person, there is no specific action to take to prevent AFM. But you can take steps to prevent getting sick from a virus.

You can lower the risk of getting a virus by:

You can lower the risk of spreading a virus by:

  • Cleaning and disinfecting frequently touched surfaces, including toys, mobile devices, and doorknobs.
  • Covering coughs and sneezes with a tissue or upper shirt sleeve, not hands.
  • Staying at home if you or your child is sick.


Medicines used in the Treatment :

Medicines used mainly to boost the immune system

  • Steroids

  • plasma exchange

  • Immunoglobulins

  • Antivirals


REFERENCE :

http://medicinenews.com

http://who.in

http://cdc.gov

http://uptodate.com

http://elsevier.in