Brain On Fire: A Rare Autoimmune Disease
A rare autoimmune encephalitis that drove neurologists stunned with its extravagant symptoms.
Ever wondered that the Netflix film “Brain on Fire” was based on the true story of Susannah Cahalan. The condition she went through was not exclusively extremely unusual, but also very complicated to diagnose at that time.
She suffered from an autoimmune disease of the brain, which is medically termed as Anti — N-methyl-D-Aspartate (Anti — NMDA) Receptor Encephalitis. After being successfully treated she released a book named “Brain on Fire: My Month of Madness” in 2012 to uncover her journey as a patient, the hardships she went through before getting the right medications, inaccurate diagnosis, and educating the public about the severity of the Anti — NMDA Receptor Encephalitis.
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What is Anti — NMDA Receptor Encephalitis
The NMDA is the glutamate receptor present on the neurons and acts as an ion transporter for the conduction of impulses to and from the brain. It moreover keeps the Dopamine levels in check, which is essential for the proper functioning of the brain.
The hypo functioning of NMDA glutamate synapse causes an increase in dopamine levels in the brain which results in Psychotic Symptoms like loss of behavior control, motor controls of the brain, concentration issues, memory loss, etc.
Dopamine dysregulation has been further linked to schizophrenia, attention-deficit hyperactivity disorder (ADHD), bipolar disorder.
Brain on fire disease is autoantibody-mediated encephalitis, which was first identified in 2007.
An autoimmune neurological disorder in which antibodies developed in the body which are supposed to attack the pathogens causing the disease starts to attack its own body and in this case antibodies binds with NMDA receptor present in the neurons of the brain and causes dysfunction and blockage in the glutamate synapse which outcomes in the disorder named as (anti — NMDA) Receptor Encephalitis.
The disease has been associated with the presence of underlying conditions like cancer, tumors (Ovarian Teratoma generally), infections (Herpes Simplex Encephalitis most commonly reported), etc.
Women and children are susceptible to this rare disease, with approximately 80 percent of cases recorded being of females and children in comparison to men.
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Symptoms of Anti — NMDA Receptor Encephalitis
The symptoms of Anti — NMDA Receptor Encephalitis occur in phases that may begin as a normal headache and rapidly modify into neuropsychiatric issues within the weeks. The signs are entirely based on the acuteness of the disease.
Various acute symptoms encountered by the patients of this ailment are described below:
- Cognitive Impairments like confusion, lack of concentration, difficulty in perceiving and comprehending thoughts, decreased level of consciousness, etc.
- Behavioral Changes like mood swings, disorganized thoughts, hallucination, paranoia, hearing voices, etc.
- Seizures
- Movement Disorders like Tardive dyskinesia (involuntary movements of the muscles of trunk, limbs, face, etc as seen in the patients with a withdrawal syndrome)
- Autonomic Instability (a life-threatening condition in which neurons regulating the involuntary muscles of the body are damaged as of heart, blood vessels, digestive system, etc)
- Comatose
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Atypical symptoms commonly found in children are as follows:
- Cerebellar ataxia (loss of muscle control and coordination due to damage in the cerebellum)
- Hemiparesis (mild but partial paralysis of one side of the body)
Treatments to cure “Brain on Fire”
Similar to the symptoms, the treatment of the Anti-NMDA Receptor Encephalitis also exists in phases. The first-line treatments include procedures to eradicate the causative agents of the disease, and second-line treatments consist of drugs to suppress the symptoms elicited due to the illness.
Some First-line treatments include:
- Steroids (Oral or Intravenous)
- Intravenous Immunoglobulins (IVIG)
- Plasma Exchange (Dialysis)
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A few notable Second-line treatments are:
- Immunosuppressants (crucial therapy to cure autoimmune disorders)
- Cyclophosphamide (reduces inflammation)
- Rituximab (a monoclonal antibody)
- Epileptic drugs to relieve the Seizures.
- Antipsychotic/Neuroleptic drugs
Besides the treatment described above, it is vital to rule out the underlying factors like strokes, tumors, intoxications that contribute to stimulating the severity of Anti — NMDA Receptor Encephalitis.
Source: https://thesecondangle.com/brain-on-fire-a-rare-autoimmune-disease/
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Tags: Autoimmune Diseasebraindiseaseencephalitisneurologists
