SCHOOL OF BIO AND CHEMICAL ENGINEERING

DEPARTMENT OF BIOMEDICAL ENGINEERING

NEURAL ENGINEERING (BME18R405)

DEMYELINATING DISEASES

PRESENTED BY
M.MARIKANI
(9916020076)
BME IV YEAR
KARE
DEMYELINATING DISEASES

 Damage to the protective covering (myelin sheath) that surrounds nerve fibres in
your brain, optic nerves and spinal cord.
 When the myelin sheath is damaged, nerve impulses slow or even stop, causing
neurological problems.
TYPES OF DEMYELINATING DISEASES

 Some myelinating diseases:

1. Multiple sclerosis
2. Optic neurites
3. Neuromyelitis optica (Devic’s disease)
4. Transverse myelitis
5. Acute disseminated encephalomyelitis
6. Adrenoleukodystrophy
7. Adrenomyeloneuropathy
MULTIPLE SCLEROSIS

 Common demyelinating disease of CNS

 Auto-immune disease
 Body’s immune system attacks and produces antibodies against its own cells
 Attacks the myelin sheath
 Injury to the myelin sheath causes inflammation of nerve fibres that surrounds it
 The process leads to multiple areas of scarring (sclerosis)
OPTIC NEURITIS

 Inflammation of the optic nerve in one or both eyes

 Can be an early sign of the MS
 Symptom: Pain and temporary vision loss
 Treatment: steroid medications and blood transfusion
NEUROMYELITIS OPTICA

 Inflammation and demyelination of the CNS, especially of the optic nerve and
spinal cord
 Optic neuritis – inflammation of optic nerve
 Myelitis – inflammation of spinal cord
 Cause for the disease is unknown
 Often perceived as MS but it is distinct
 Symptoms: blindness, paralysis, painful spasms, loss of sensation, uncontrollable
vomiting and hiccups and bladder dysfunction from spinal cord damage
TRANSVERSE MYELITIS

 Loss of myelin in spinal cord

 Cause for the disease is unknown
 Symptoms: lower back pain, bladder and bowel problems, numbness in toes,
fatigue, sensitivity to touch
 No cure for this disease
 Steroid shots or plasma exchange (PLEX) may bring down the swelling in spinal
cord
ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)

 Damages myelin in the brain and spinal cord

 Auto-immune disease
 May be caused due to bacterial or viral infection
 Symptoms: fever, headache, nausea, eyesight problems
 In rare cases, ADEM can be deadly
ADRENOLEUKODYSTROPHY (ALD)

 Disease linked to the X chromosome

 Occurs primarily in males especially children between ages 4 and 10
 Causes VLCFAs accumulation in the brain
 Resulting in destroy of myelin sheath, responsible for brain function
 Symptoms: aggression, poor memory, difficulty in reading and writing and
understanding speech, vision problems, seizures, deafness
 Without treatment, ALD progresses rapidly, leads even to death
SCHILDER’S DISEASE

 Affects boys between ages 7 and 12

 Wears away the brain and spinal cord
 Can affect breathing, heart rate and blood pressure
 Hard to predict
 Symptoms: head ache, fever, seizures, vision problems, change in personality
 Symptoms are manageable with steroids and drugs that calm the immune system
ADRENOMYELENONEUROPATHY (AMN)

 It is a form of X-linked ALD

 AMN is caused by mutation in ABCD1 gene
 ABCD1 gene gives the body instructions to make the adrenoleukodystrophy
protein (ALDP)
 ALDP helps in transport of VLCFAs into peroxisomes
 Peroxisomes helps to break down fats
 Symptoms: difficulty in walking, ataxia (impaired coordination), hypertonia
(muscle overactivity), adrenal insufficiency, impotence
 Treatment: steroid replacement therapy for adrenal insufficiency
BALO’S DISEASE ( CONCENTRIC SCLEROSIS)

 Rare form of MS
 It affects adults more often than children
 Symptoms: headache, memory loss, muscle spasms, seizures
 Treatment: steroids can bring down the swelling in your brain and spinal cord
CHARCOT-MARIE-TOOTH DISEASE (CMT)

 It affects PNS
 Inherited from parents
 Symptoms show up in early adult years
 Symptoms: weakness in legs, ankles and feet, trouble in walking or running
 Treatment: physical and occupational therapy, exercise helps in recovery
HTLV-1 ASSOCIATED MYELOPATHY (HAM)

 Results from a virus called HTLV-1.

 Leads to swelling of brain and spinal cord
 Symptoms: constipation, double vision, deafness, tremors
 Treatment: steroids can help ease the symptoms
THANK YOU