It is also called acute inflammatory demyelinating polyradiculoneuropathy (AIDP). It is a neurological disorder in which the body's immune system attacks the peripheral nervous system, the part of the nervous system outside the brain and spinal cord. The onset of GBS can be quite sudden and unexpected and requires immediate hospitalization. It can develop over a few days, or it may take up to several weeks with the greatest weakness occurring within the first couple of
weeks after symptoms appear.

It often develops after a respiratory or gastrointestinal viral infection, surgery, injury, or a reaction to an immunization.

In GBS the immune system starts to destroy the myelin sheath that surrounds the axons of many nerve cells, and, sometimes, the axons themselves. When this occurs, the nerves can’t send signals efficiently, the muscles lose their ability to respond to the commands of the brain, and the brain receives fewer sensory signals from the rest of the body. The result is an inability to feel heat, pain, and other sensations.

Note/ facial nerve is the most commonly involved nerve.

At first symptoms begin as weakness or tingling sensations in the legs which sometimes spreads to the arms and upper body. A neurological exam usually reveals a loss of all deep tendon reflexes “Areflexia”

Unique signs and symptoms to GBS:

- Symptoms on both sides of the body (symmetrical)

- Symptoms appear quickly, days or weeks instead of months Loss of reflexes (rapid progressive illness)

- High protein level in cerebral spinal fluid -by lumbar puncture- ( CSF protein levels correlate with the number of demyelinating electrophysiologic criteria abnormalities, starting from > 45 mg/dl for those who meet one criteria of demyelination).

The goal of treatment is to prevent breathing problems and relieve symptoms, in addition to immune system suppressing by Plasmapheresis (a procedure used to remove the plasma and replace it with other fluids. Antibodies are also removed with the plasma), and IVIG (intravenous immunoglobulin), other supportive therapies like hormonal and physical to increase muscle flexibility and strength.

هذا أعراضه كلش nonspecific
لغرض التبسيط، فهي أما تكون cardiovascular (wet beriberi)
أو تكون neurological (dry beriberi) أو مزيج من الإثنين

• while Guillain-Barré syndrome (GBS) is an important cause of acute flaccid paralysis, thiamine deficiency should be considered an important differential diagnosis, particularly in patients with a history of dietary deprivation and incarceration. 

• high-output cardiac failure should prompt investigation of thiamine deficiency as a cause.

Case 1:

72 year-old male with a history of diabetes and remote Whipple procedure who presented with four weeks of weakness and sensory loss. Exam showed distal sensory loss, mild proximal and distal lower limb weakness, and reduced reflexes. Axonal neuropathy was noted on EMG. CSF showed cytoalbuminologic dissociation. IVIG was initiated for a presumptive diagnosis of GBS. He subsequently developed hypotension requiring pressors, lactic acidosis, encephalopathy, ocular muscle weakness and respiratory failure.

Case 2:

38 year-old male with a history of alcoholism and Crohn's was transferred to the facility with 3-4 weeks of progressive sensory and motor deficits, hypotension, tachycardia, and lactic acidosis attributed to GBS and superimposed sepsis. He subsequently developed severe generalized weakness, respiratory failure, encephalopathy and extraocular muscle paresis. Workup revealed axonal neuropathy, no infection and normal CSF findings.

Diagnosis/Treatment:

In both cases, features of Wernicke's and wet beriberi prompted the clinical suspicion supported by brain MRI studies and reduced thiamine levels (19&23 nmol/liter; normal 80-150nmol/liter). Hypotension and cardiac failure rapidly resolved with thiamine repletion. Ocular muscle weakness and encephalopathy resolved within days, but limb weakness only partially improved.

Normal Brain MRI

In conclusion
A high index of suspicion for thiamine deficiency in patients presenting with progressive neuropathy is important as there is significant overlap with GBS. Development of hypotension, tachycardia, lactic acidosis, mental status changes, or extraocular muscle paresis in cases of possible GBS should prompt consideration of thiamine deficiency.

بعد أكو شغلة

تحملوني

Researches showed that thiamine deficiency is associated with neurological problems, including cognitive deficits. Multiple similarities exist between classical thiamine deficiency and Alzheimer's disease (AD) in that both are associated with cognitive deficits and reductions in brain glucose metabolism. Thiamine-dependent enzymes are critical components of glucose metabolism that are reduced in the brains of AD patients and by thiamine decline, and a decrease in their levels could account for the reduction in glucose metabolism. In preclinical models, reduced thiamine can drive AD-like abnormalities, including memory deficits, neuritic plaques, and hyperphosphorylation of tau. Furthermore, excess thiamine diminishes AD-like pathologies. In addition to dietary deficits, drugs or other manipulations that interfere with thiamine absorption can cause thiamine deficiency. Elucidating the reasons why the brains of AD patients are functionally thiamine deficient and determining the effects of thiamine restoration may provide critical information to help treat patients with AD.

شوفوا هذه الكيس

لاحظ هاوس گللهم انطوها thiamine وبعدين من يتعدل انطوها كيك او ايس كريم، لأن الدماغ يحتاج الـ thiamine بالـ metabolism فإذا ما عوضته أول شي، رح يزيد الـ damage.

في سياق متصل بما انو البارحة امتحنت توليد، وحدة من الـ changes اللي تصير خلال الحمل هي Hyperemesis Gravidarum, فالمريضة رح يصير عدها thiamine deficiency، همين بالـ management ما أنطي glucose اذا ما أعوض الـ thiamine.