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Guillain-Barré (ghee yan-bah ray) (GBS), Chronic Inflammatory Demyelinating Polyradiculoneuropathy (C.I.P.D.), Miller Fisher and other syndromes are rare and can make a person's very weak very quickly. These syndromes, are disorders that consists of weakness and  paralysis of many parts of the body, along with abnormal sensations. The illness presents in several ways, at times making the diagnosis difficult in the early stages. The specific cause is a autoimmune reaction. Research indicates that, the nerves of the person who has Guillain-Barré or a related syndrome, are attacked by the body's defense system against disease (antibodies and white blood cells). As a result of this attack, the nerve insulation (myelin) and sometimes even the covered conducting part of the nerve (axon) is damaged. This attack delays & changes of the nerve "messages", between the sender (the brain) and receiver (muscle). The abnormal sensations and weakness quickly follow. The affected individual is crippled.

Treatment is different based upon the cause. Which can be a infection bacterial, viral, Vaccination, Surgery, Trauma or chemical exposure. The immune reaction to vaccination, surgery, stress or injury  triggers the disease.

Lets say a person gets GBS after a flu shot. Give IVIg or steroids right away.

One gets GBS after IVIg then give prednisone or chemotherapy.

You get GBS after a stomach illness, take a antibiotic.

If you cannot find a treatment please contact us.

We supply treatment protocols to doctors and Hospitals and are all described in our e-book called, "the flame within". This E-Book is also packed with information on how to prevent GBS and all autoimmune diseases.

In the old days before immunotherapy the patients who had GBS would lay in the hospitals units for 6 months or more on ventilators. These days patients can get treatment in a few days and they are out the door from the hospital. Things have improved but the incidence of this disease has gone up rapidly.

What is Miller Fisher Syndrome:

In MFS syndrome the patient presents with eye movement disorders, weakness . Usually the person has no eye movements. Treatment of Miller Fisher is the same as for GBS or CIDP.

Imran Khan MD

Nanotech Medical Center Wapda Town Lahore

LANDRY'S ASCENDING PARALYSIS (1859)

The sensory and motor systems may be equally affected. However the main problem is usually a motor disorder characterized by a gradual diminution of muscular strength with flaccid limbs and without contractures, convulsions or reflex movements of any kind. In almost all cases micturition and defecation remain normal. One does not observe any symptoms referable to the central nervous system, spinal pain or tenderness, headache or delirium.

The intellectual faculties are preserved until the end. The onset of the paralysis can be preceded by a general feeling of weakness, pins and needles and even slight cramps. Alternatively the illness may begin suddenly and end unexpectedly. In both cases the weakness spreads rapidly from the lower to the upper parts of the body with a universal tendency to become generalized.

The first symptoms always affect the extremities of the limbs and the lower limbs particularly. When the whole body becomes affected the order of progression is more or less constant: (1) toe and foot muscles, then the hamstrings and glutei, and finally the anterior and adductor muscles of the thigh; (2) finger and hand, arm and then shoulder muscles; (3) trunk muscles; (4) respiratory muscles, tongue, pharynx, esophagus, etc. The paralysis then becomes generalized but more severe in the distal parts of the extremities. The progression can be more or less rapid. It was eight days in one and fifteen days in another case which I believe can be classified as acute. More often it is scarcely two or three days and sometimes only a few hours.

When the paralysis reaches its maximum intensity the danger of asphyxia is always imminent. However in eight out of ten cases death was avoided either by skilful professional intervention or a spontaneous remission of this phase of the illness. In two cases death occurred at this stage . . . When the paralysis recedes it demonstrates the reverse of the phenomenon which signaled its development. The upper parts of the body, the last to be affected, are the first to recover their mobility which then returns from above downwards.

Jean Baptiste Octave Landry de Thezillat (1826-1865)

Distinguishing acute-onset CIDP from Guillain-Barre syndrome with treatment related fluctuations.

    Ruts L, van Koningsveld R, van Doorn PA.

 Department of Neurology, Erasmus MC, Rotterdam, The Netherlands.

    Guillain-Barre syndrome (GBS) patients may worsen after initial

treatment (treatment-related fluctuation [TRF]). It is difficult to

distinguish GBS-TRF from chronic inflammatory demyelinating

polyneuropathy with acute onset (A-CIDP). The authors compared 13

patients with A-CIDP with 11 patients with GBS-TRF and concluded that

A-CIDP should be suspected when a patient with GBS deteriorates after 9

weeks from onset or when deterioration occurs three times or more.

Maintenance treatment should then be considered.

A-42-year old man presented with progressive difficulty in walking and climbing stairs for past 2 days, weak handgrip and inability to raise arms above shoulder level for the past 1 day. He developed intermittent choking on swallowing liquids since the morning of the day of admission. We discuss here Guillain Barre Syndrome as an acute immune mediated polyneuropathy.

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(This report is here to show that some people may benefit from steroids)  If a treatment is not helping then think of alternatives)
 

Case report

A-42-year old man presented with progressive difficulty in walking and climbing stairs for past 2 days, weak handgrip and inability to raise arms above shoulder level for the past 1 day. He developed intermittent choking on swallowing liquids since the morning of the day of admission. There was no history of facial asymmetry, bladder or bowel involvement. At the time of admission, he was conscious, alert and oriented, afebrile, PR=70/min and BP=130/76 mm Hg with no postural drop. CNS examination revealed a hypophonic speech, bilateral sluggish gag and palatal reflexes, generalized hypotonia, weak neck and abdominal muscles. Power was 2/5 MRC grade in upper limbs with markedly weak grip bilaterally and 4/5 in the lower limbs with absent deep tendon reflexes and bilateral flexor plantars. Tactile sensation was impaired by 10-15% in hands and feet. CSF showed 2 WBC (100% lymphocytes), glucose=52mg/dl and protein=66mg/dl.

A clinical diagnosis of AIDP was made and patient initiated on IVIG immediately after admission. However, next day he was found to have bilateral facial weakness, R>L. On the third day of IVIG he complained of an increase in choking episodes and motor power in the lower limbs had decreased to 3/5. A Ryles tube was inserted for feeding and patient made nil per orally. On day 4 he developed mild difficulty in breathing with a respiratory rate of 24/min, a clinically clear chest and a normal chest X-ray. FVC decreased from 3L at the time of admission to 2.1 L. In view of progressive deterioration and impending respiratory failure despite IVIG therapy he was started on intravenous methyprednisolone, 1000mg Q day. On the very next day his breathlessness decreased and he reported a subjective increase in strength in all four limbs. Over the next 24 hours an objective increase in lower limb power to 4/5 and an increase in hand grip bilaterally was found. Over following 3-4 days he was able to accept liquids and solids orally without choking. He showed consistent improvement and was discharged after a stay of nearly a month in the hospital. At the time of discharge he was able to walk without support and had good handgrip bilaterally.

Discussion

Guillain Barre Syndrome is an acute immune mediated polyneuropathy. Although most patients begin to recover spontaneously within 2 weeks after maximum weakness is reached, symptoms which range from fatigue to complete paralysis may persist in some cases .

Several conflicting reports have been published regarding role of steroids in AIDP. One study revealed beneficial effect of a combination of IVIG with methylprednisolone while another recently published study refuted any beneficial effect of addition of methylprednisolone to IVIG.

In our patient, nerve conduction studies done on the second day after admission, that is day 3 of illness revealed absent H reflex, prolonged F wave latency and distal latencies, decreased CMAP amplitude in all motor nerves, absent bilateral median and ulnar sensory conduction velocities and normal sural nerve response. It also showed slowed motor conduction velocities and conduction blocks. These latter findings which are only seen in 10-20% of GBS patients early after onset of illness are however one of the important diagnostic criteria of CIDP an illness closely related to AIDP which shows a beneficial response to steroids. Hence it seems that a subset of patients who reveal these findings on NCS early in the course of illness may be the ones showing a beneficial response to steroids, specially a synergistic effect when used with IVIG ^{Combined therapy of intravenous immunoglobulin and methylprednisolone in patients with Guillain-Barré syndrome: the results of a multicentre double blind placebo controlled clinical trial. Peripher Nerv Syst2001; 6:186-7.', 200);" onmouseout="tooltip.hide();">6}. Further we suggest that steroids may be tried in IVIG failed cases of GBS who continue to progress rapidly.

Address for Correspondence

Nitin. K. Sethi, M.D.
Chief Resident, Neurology
Saint Vincent's Hospital and Medical Center
153 West, 11th Street
New York, NY 10011

 

 

 

Pathalogy in CIDP and autoimmune diseases