Gullian Barr Syndrome – A Rare Disease Case Report

 

Shaik Asha Begum1,3*, T. Vinay Kumar1, Shaik Abdul Rahaman4, S. Joshna Rani3,

Sreya Kosanam1, Y. Veena1

1Department of Pharmacology and Pharmacy Practice, Nirmala College of Pharmacy,

Atmakur, Mangalagiri, AP, India – 522503.

2 Pharm D Students, Nirmala College of Pharmacy, Atmakur, Mangalagiri, AP, India - 522503

3IPT, SPMVV, Tirupati, AP, India – 517501.

4Princiapl and Professor, Nirmala College of Pharmacy, Atmakur, Mangalagiri, AP, India – 522503.

*Corresponding Author E-mail: sk.asha86@gmail.com

 

ABSTRACT:

Guillain-Baree syndrome also called guillain–Barré–Strohl syndrome, Landry's paralysis, post - infectious polyneuritis whichis a rare, at one or two cases per 100, 000 people every year, where auto-immune disorder in which a persons own immune system damaged the nerves, causing muscule weakness and sometimes paralysis. GBS can cause symptoms that last for a few weeks to several years. Most people recover fully, but some have permanent nerve damage. some people have died of GBS. Several things are known to tigger GBS. About two-thirds of people with GBS had diarrhea or a respiratory illness several weeks before developing symptoms. Infection with campylobacter jejuni, which causes diarrhea, is one of the most common risk factors for GBS. People can develop GBS after some other infections, such as flu, cytomegalovirus, Epstein barr virus, and zika virus. Very rarely, people have developed GBS in the days or weeks after receiving certain vaccines. It can be caused by campylobacter infection that is diagnosed when a laboratory tests detect campylobacter bacteria in stool, body tissue, or fluids. The test could be a culture that isolates the bacteria or a rapid diagnostic test that detects genetic material of the bacteria. The major aim of this case report is to present a classic case of this condition, to highlight an awareness of differing treatment options, and to advocate referral to a given its physician potential severity. Treatment for GBS including antibiotics, drink extra fluids as long as diarrhea lasts, plasma exchange, immunoglobulin therapy2.

 

KEYWORDS: Immune response, nervous system, immunoglobulins, plasmapheresis, Miller Fisher syndrome.

 

 


INTRODUCTION:

Guillain –barre syndrome is a rare disorder in which bodys immune system attacks your nerves. Weakness and tingling in extremities are usually the first symptoms. These sensations can quickly spread, eventually paralyzing whole body. The exact cause is unknown but typically trigerred by infection, by surgery and rarely by vaccination.

 

Theres no known cure for GBS, but several treatments can ease symptoms and reduce the duration of illness2. Most of the people recover from GBS. Mortality rate is 4-7%, between 60-80% of people are able to walk at 6 months. During the acute phase, the disorder can be life threatening, with about 15% of people developing weakness of the breathing muscles and thereby requiring mechanical ventilation. Some are affected by changes in the function of the autonomic nervous system, which can lead to dangerous abnormalities in heart rate and blood pressure3.

 

Patient Information, Clinical Findings, Timeline:

A 11 years old male patient was admitted in the hospital with the chief complaints of weakness of both lower limbs associated with difficulty in walking, pain in legs which is progressively increased. Mild parasthesias in both legs, fever, gait abnormality4.

 

Diagnostic Assessment:

The patient is in a conscious and in a coherent state, with Afebrile temperature, and weakness in lower limbsand in CNS paraparesis both lower limbs and reflexes are observed. Haematological tests, blood levels are less than the normal values. remaining all vitals are normal.

 

Therapeutic Interventions:

He has no previous past history. patient admitted in hospital, doctors suggested methyl prednisolone with inj. IV Immunoglobulins and antibiotics.

 

Follow-Up And Outcomes:

According to all the Laboratory profiles, mentioned above, the patient has been diagnosed with guillian barres syndrome a rare disease and physicians prescribed the following medications in order to control or to prevent the further complications of the disease.

 

DISCUSSION:

The syndrome is named after the French neurologists Georges Guillain and Jean Alexandre Barré, who, together with French physician André Strohl, described the condition in 1916. Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness that can be caused by the immune system damaging the peripheral nervous system. GBS affects both sides of the body, and the initial symptoms are changes in sensation or pain often in the back along with muscle weakness, beginning in the feet and hands, often spreading to the arms and upper body. The symptoms may develop over hours to a few weeks. The diagnosis is usually based on the signs and symptoms through the exclusion of alternative causes and supported by tests such as nerve conduction studies and examination of the cerebrospinal fluid5. the underlying mechanisminvolves an autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nerves and damages their myelin insulation. In severe weakness, treatment with intravenous immunoglobulins or plasmapheresis, together with supportive care, will lead to good recovery in the majority of people. Recovery may take weeks to years, with about a third having some permanent weakness6.

 

These are mainly 3 types:

·       Acute inflammatory demyelinating polyradiculoneuropathy (AIDP),

·       Miller Fisher syndrome (MFS),

·       Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN)

 

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): The most common form in North America and Europe. The major sign is muscle weakness that starts in the lower part of body and spreads upward.

 

Miller Fisher syndrome (MFS): where paralysis starts in the eyes. MFS is also associated with unsteady gait. MFS is less common in the U. S. but more common in Asia.

 

Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN): are less common in the U. S. but AMAN and AMSAN are frequent in China, Japan, and Mexico7.

Treatment

·       Intra-venous Immunoglobulin for 5 days,

·       Plasma exchange (200-250ml/kg) for 5 sessions.

·       Plasmapheresis, steroids, immunosuppresants are alternatives, if IVIG is ineffective.

·       Combined administration of immunoglobulin and interferon is effective in some patients.

·       Eat green vegetables, citrus fruits which are rich source of antioxidants that help to boost your immunity. Take fiber rich diet. Take vitamin B rich foods which helpful for healthy nerves in brain 8.


 

S. No

Trade Name

Generic Name

R. O. A

Dose

Frequency

1.

Carimune nanofiltered

Immune globulin HUMAN (IGG)

IV

3g

Once a day

2.

Immunoglobulin

-

IV

0. 4g/kg

Once a day

3.

plasmapheresis

-

IV

200-250ml/kg

Once a day

4.

Inj, methyl prednisolone

-

IV

500mg

Once a day

 

CONCLUSION:

Overall, GBS is potentially a rare disease and can be treatable under the appropriate level of care. The major aim of this case report is to highlight the several treatment options, to bring awareness about the disease and to advocate referral to neuralagist given its potential severity.

 

ETHICAL COMMITTEE APPROVAL:

Obtained approval from the Manipal hospital ethical committee. We also obtained informed consent from the patient which we have attached with this case report for the journal’s reference.

 

REFERENCES:

1.      Avinash. B. Thalkari, Pawan. N. Karwa, Chandrakant S. Gawli. Anonychia Congenita: A Rare Disease. Res. J. Pharmacology and Pharmacodynamics. 2020; 12(2): 55-56. doi: 10. 5958/2321-5836. 2020. 00011. 7

2.      Tejasavi Mahajan, Navdeep Singh, Kamya Goyal, Shammy Jindal, Vinay Pandit, M. S. Ashawat. Recent Updates on Psoriasis: A Review. Asian Journal of Pharmaceutical Research. 2022; 12(1):76-3. doi: 10. 52711/2231-5691. 2022. 00012

3.      Girish Yogesh Pawar, Rushikesh Rakesh Chaudhari, Tushar Naresh Sonawane, Jayesh Shyam Desale, Suraj K. Ishikar. A Short Review on Study on Different Clinical Trials Performed on Drugs before it Comes into The Market. Asian Journal of Pharmaceutical Research. 2022; 12(1):84-7. doi: 10. 52711/2231-5691. 2022. 00013

4.      Ganesh G. Dhakad, Sangita P. Shirsat, Kaveri P. Tambe, Vinit Kairnar, Ritik. S. Jain. Review on Immuno-Oncology agents for Cancer Therapy. Asian Journal of Pharmaceutical Research. 2022; 12(1):110-5. doi: 10. 52711/2231-5691. 2022. 00017

5.      Santhosh Kumar V, Praveen D. Sugar and Chronic Diseases. Research J. Pharm. and Tech. 2016; 9(6): 650-654. doi: 10. 5958/0974-360X. 2016. 00123. 2

6.      Sukhbir Lal Khokra, Bharat Parashar, Hitesh Kumar Dhamija, Manju Bala. Immunomodulators: Immune System Modifiers. Research J. Pharm. and Tech. 5(2): Feb. 2012; Page 169-174. doi: Not Available

7.      Lippi G, Plebani M. Biomarker research and leading causes of death worldwide: a rather feeble relationship. Clin Chem Lab Med 2013;51:1691-3. 10. 1515/cclm-2013-0210 [PubMed] [CrossRef] [Google Scholar]

8.      Tong N. Priority Diseases and Reasons for Inclusion. In: Priority Medicines for Europe and the World 2013 Update. World Health Organization Publisher, Geneva, Switzerland, 2013. [Google Scholar]

 

 

 

 

Received on 30.03.2022         Modified on 10.05.2022

Accepted on 28.06.2022     ©A&V Publications All right reserved

Res.  J. Pharmacology and Pharmacodynamics.2022;14(4):234-236.

DOI: 10.52711/2321-5836.2022.00040