Cerebral palsy with Dandy-Walker Syndrome
Cerebral means a portion of brain that is affected and palsy refers to complete or partial paralysis of muscles. Cerebral palsy is a hypernm that indicates abnormal activities of motor neurons which ceases the further brain development. It also affects the body tone and posture which is a result of brain lesions. Cerebral palsy is a disorder caused during pregnancy, delivery, or shortly after birth upto age3. Without sufficient oxygen and nutrients, brain cells are injured and killed. An ischemic blood circulation causes disruption in the energy producing mitochondria of the neurons.
Dandy Walker syndrome is a genetic sporadic condition that is present since birth and is a cause of increased cerebrospinal fluid in the brain.The condition is described by partial or complete absence of a part of brain located between the two cerebellar hemispheres.
Signs and symptoms of cerebral palsy with Dandy Walker Syndrome
- Variations in muscle tone, such as being either too stiff or too floppy
- Stiff muscles and exaggerated reflexes (spasticity)
- Stiff muscles with normal reflexes (rigidity)
- Jerky eye movements
- Lack of muscle coordination (ataxia)
- Tremors or involuntary movements
- Slow, writhing movements (athetosis)
- Delays in reaching motor skills milestones, such as pushing up on arms, sitting up alone or crawling
- Favouring one side of the body, such as reaching with only one hand or dragging a leg while crawling
- Difficulty walking, such as walking on toes, a crouched gait, a scissors-like gait with knees crossing or a wide gait
- Excessive drooling or problems with swallowing
- Difficulty with sucking or eating
- Delays in speech development or difficulty speaking
- Difficulty with precise motions, such as picking up a crayon or spoon.
- Blood tests
- CT scan of the head
- Hearing screen
- MRI of head
- Vision testing
Recently, the introduction of stem cells into the field of health sciences has raised the hopes for treatment in neuromuscular and neurological diseases. Stem cells are believed to exist in all tissues in human body and may be totipotent, pluripotent, multipotent depending on tissue type.
Stem cells are mainly classified as Adult stem cells and Embryonic stem cells. They are further classified according to the way they are derived and the source tissues like, Mesenchymal stem cells (MSCs), Haematopoietic stem cells (HSCs) and induced pluripotent stem cells (iPS) cells are the major types of stem cells.
We use MSCs and HSCs that are multipotent, self renewing cells derived from adult tissues that can form a number of cells or tissues that are usually restricted to a particular germ layer. The major advantage of MSCs and HSCs are the ease of harvest and lack of immunoreactivity since they are derived from autologous sources. Autologous stem cells are derived from an individual’s own body, and can be obtained from bone marrow, adipose tissue and peripheral blood. These are processed in tissue culture lab to isolate stem cells which are then used for therapeutic purposes and are then given in the form of doses to the patient.
A cell suspension from immature nervous and haematopoietic tissues is injected into the subarachnoidal space of patient through a spinal puncture (Intrathecally) that can considerably enhance reparative capacities of the nervous tissue and improve the function of damaged brain. This technique is an effective, safe and immunologically proved method of therapy for patients of cerebral palsy with Dandy walker syndrome.
Post stem cell therapy includes occupational therapy, physiotherapy, speech therapy followed by proper diet plan.
Case Study Cerebral Palsy:
A 03 year old baby girl admitted to our hospital for treatment of Cerebral Palsy with Dandy walker syndrome.
Presenting complaints: Unable to walk or stand properly without support, unable to hold neck, twisted extremities, birth history full term normal delivery.
Associated complaints: pathological jaundice 3 days after birth, convulsions since the age of 11 months, last convulsions 15 days before with severe spasm.
Delayed milestones started at the age of 11 months.
Physical examination: Musculoskeletal deformity in hams B/L walks with support, weak reflexes.
Treatment Plan: In cerebral palsy, there is brain tissue damage during pregnancy, delivery or shortly after delivery accompanied by;
- Impaired movement, speaking, hearing, eyesight.
- Lack of co-ordination and mental underdevelopment.
Stem cells provide the most feasible treatment for CP due to their immense regenerative capacity and their ability to differentiate into any type of cell, depending on their surrounding microenvironment. The objective of stem cell therapy is to repair the injured areas.
Here, the stem cell therapy protocol devised was as follows:
- Mesenchymal Stem Cell Transplant:
Bone Marrow & Adipose Tissue derived Stem Cells with Stromal Factor transplanted:
- Intrathecally via lumbar puncture supplemented with,
- Intravenous route.
- Supplementation of deficient vitamins like D3, B12 ,and minerals like zinc, calcium; amino acids like glutathione, arginine and proteins so as to maintain the micro environment needed for stem cell to differentiate and maintain the potency.
- Regular Physiotherapy exercises
Patient showed good improvement symptomatically
Speech improvement (clarity in speech) seen.
Walking only with support of walker, able to climb stairs with minimal help.
Motor reflexes improved
Social- Psychological improvement is observed.
Increase in appetite.
The patient is still under observation and follow up.