Nerve Disease

Guillain-Barré Syndrome / Acute Myeloradiculoneuritis
Short Summary

45-year-old male who presented with acute onset of flaccid paraparesis more pronounced on the left, sensation of current shocks in both legs and sensory level from the umbilicus downwards. The symptoms have reached their pick within less than a day. On examination the left lower limb was plegic and the right lower limb was partially weak. EMG was interpreted as demyelinating sensorimotor neuropathy. MRI showed hyperintense signals at the dorsal terminal segment of the marrow consistent with myelitis. Treatment with plasmapheresis and IV steroids was followed by improvement in the right leg weakness. The patient was later treated with IVIG. He reported improvement during the first year which was then followed by stabilization of his condition.

Patient's Questions

1. Is this diagnosis correct? Or could it be something else?

2. Can you suggest any alternative treatment?

3. Is there any relevant recovery threshold?

4. I am currently walking with two crutches. Am I supposed to use them forever or is there any chance I can get rid of them?
 

Medical Background

Patient: male, 45 years old, Italy
Diagnosis: Guillain-Barré Syndrome

In anamnesis:
- tonsillectomy at 5 years;
- left lumbalgia and lumbosciatic for which he underwent a lumbar MRI in 1997 with finding of small L4-L5 protrusion in the left paramedian area, and in L3-L4 the disk globally goes beyond the borders with no hernia lesions.
On 03.2005, the patient was at work. At around 10 a.m., he suddenly felt heavy current shocks along his lower limbs and simultaneously an intense sense of weakness on both legs, making him no longer able to stand up. At about 12 noon, due to the persistence of these disorders, the patient was forced to leave the workplace and went home, hoping that the trouble might disappear. During the afternoon, there was no improvement. The lower limbs reached a partial insensitivity with severe difficulties to stand up. At about 9 p.m., with the help of his wife the patient tried to go to bed on the upper floor. Nevertheless, while on the stairs, he felt a severe pain on his legs and immediately afterwards he lost sensitivity from the umbilicus downwards. Despite from this he laid on the bed. The following morning the situation had not improved. The patient decided to go to the emergency unit of the Treviso "Ca’ Fondello" Hospital where the doctor on duty immediately ordered his hospitalization in the neurology department.

At admission in the ward the patient was not able to stand up and was examined in bed. The neurological examination highlighted: plegia of the left lower limb. The bending of the leg on the thigh on the right was possible, with minor right dorsiflexion of the foot. Strength of the upper limbs within normal limits. Hypotonia on the left lower limb. He could not reach the Mingazzini position No. 11. No DTR on the lower limbs. Identical bilateral plantar reflex. Sensitivity uncompromised (he reported distal paresthesias on the lower limbs). No signs of meningeal irritation. Intact cranial nerves.

The patient underwent an urgent EMG (03. 05) with the following result: “The finding is indicative of a demyelinating sensory and motor neuropathy on the lower limbs. The examined nerve trunks on the left upper limb are within normal limits.”

Then, a lumbosacral spine MRI was performed (03.05): “first-degree anterior lystesis of L3 on L4. The L3-S1 disks show reduced thickness and dehydration. The L3-L4 disk shows right paramedian hernia with impression on the homolateral root. The L4-L5 disk shows median hernia. The L5-S1 disk shows a minor median protrusion. The conus is at place but slightly hyperintense: the diagnosis must absolutely be completed with contrast medium”.

Final dorsal-lumbosacral MRI with contrast medium (03.05): “The exam shows a more evident hyperintense alteration of dorsal marrow on the terminal segment of the medullary cone at D11-L1 level, posteriorly. The contrast medium shows a slight enhancement of the left lateral sectors. The picture first hypothesizes for myelitis”.

On 03.05, the patient also underwent a lumbar spinal tap and CSF test: clear and colourless aspect; cells 2 mm^3; Pandy reaction +---; Proteins 63 mg/dL with n.v. 15-45; Glucose 55 mg/dL with n.v. 30-70.

Due to suspected Guillain-Barré syndrome, a plasmapheresis therapy was performed. It was well tolerated by the patient (5 sessions) with complete recovery of the right lower limb mobility. Due to possible myelitis (according to the radiological data), a steroid therapy was administered in IV boluses (Solumedrol 500 mg for 5 days). It was well tolerated by the patient.

At the end of the treatments, the following exams were performed: -CSF test: confirming the albumin-cytologic dissociation. Clear and colourless aspect; Pandy's reaction +++-. Cells 1 mm^3; proteins 147 mg/dL with v.n. 15-45; glucose 57 mg/dL with n-v. 30-70. Research through PCR on cerebrospinal fluid of DNA HSV 1-2 negative, Toxoplasma gondii (gene B1) negative.

-Cervical-dorsal-lumbar MRI with contrast medium (03.05): compared with the previous one performed on March: the picture appears to be improved, with the reduction of the signal alteration affecting the marrow on the terminal segment, at D10-D12 level posteriorly. After contrast medium still posterior enhancement at D12 level and presence of some cauda roots. Unco-disk-arthrosis between C5-C6 with posterior osteophytosis beaks and intersomatic disk with central protrusion. The radiological picture was discussed with our neuro-radiologist colleagues. The reported enhancement, particularly at roots level, is sometimes also registered in case of polyradiculoneuritis and subsequently to root inflammation.

From 03. to 04.05, the patient was treated with an Ig Vena cycle (35 g for 5 days corresponding to 0.4 g/kg body weight). The therapy was well tolerated with an initial and progressive improvement of the left lower limb too, with possible bending at knee-joint level and dorsiflexion of the foot. The patient, though partially autonomous in his movements, was not able to stand up yet.

During the hospitalization, the following tests were also performed:

- Complete blood count: within normal limits, except for WBCs 10.26 thousand/mm’3. These values were also confirmed later but during the steroid treatment. Within normal limits, on the last test on 04..05, except for RBLs 4.51 million/mm’3.

- Routine blood chemistry screen: Within normal limits, except for CPK 22 ng/mL with n.v. 0-4.4; CPK 713 U/L; Fibrinogen 70 mg/dL (121 during the following control and then 181; 28 U/L on the last control on 04.06)
- Serum protein electrophoresis: Within normal limits except for alpha2 zone 13.3% (with n.v. 5.3-10.8); beta1 zone 4.3% (con n.v. 4.5-8); beta2 zone 3.3% (with n.v. 3.5-6); gamma zone 9.9% (with n.v. 10.4-19.8).

- Serum immunoelectrophoresis: monoclonal components absent.

- Urine test: within normal limits.

- Neurotropic virus serology research: Negative for anti-enterovirus; anti-Mycoplasma pneumoniae; anti-VZV, Borrelia B., anti-CMV and HSV 1-2 and Toxoplasma negative anti-IgM and positive anti-IgG.

- Preputial tampon: negative Candida investigation.

- Plasma viscosity: 0.02892 poise (with n.v. 0.014-0.018).

- Infectious disease screening (before Ig Vena treatment): Negative for HIV 1-2, anti-HCV, HbsAg, and HAV.

- ECG: within normal limits.

- Venous echo-doppler lower limbs (03.05): no echo-color doppler signs of femoral-popliteal and sural DVT bilaterally.

- Cerebral CT scan (performed before the spinal tap): no densitometrically evident foci lesions.

Therefore, the patient was discharged with the following diagnosis: “Guillain-Barré Syndrome” with the following therapy:

- Clexane 4000 UI sc (8 a.m.)
- Neurontin 300 1 tablet x 2 (8 a.m. – 8 p.m.)
- Mepral 20 1 tablet in the evening -Stilnox 1 tablet in the evening.

Today, after four years from that episode, the patient makes use of two crutches to walk and still undergoes physiatric visits with relative prescription of both individual and group physiotherapy, respectively in the gym and in the water, at the A.R.E.P. Onlus centre in Villorba (TV). Today, the patient’s drug therapy is only composed of Ig Vena (60g/day for two days), cyclically repeated at the DH of Treviso Hospital, and of steroid therapy.

Accordingly, here follows the latest discharge letter by the above mentioned DH, dated 05. 2009: “Today we are discharging the patient, formerly hospitalized for polyradiculoneuritis, who entered this department's day-hospital on 05.09 for his Ig Vena therapy cycle (60g/day for 2 days). The patient showed a stable clinical picture with asthenia and lassitude worsening. The neurological test does not show any change from the previous one. The result is: Left lower limb global hypotrophy; lack of muscle strength QF (4/5), BF (3/5) and IP (3/5) on the left. Torpid DTR left lower limb. The rest is within normal limits.

Before the hospitalization, the patient carried out:

- Complete blood count: within normal limits;

- Routine blood chemistry screen: within normal limits, except for CPK 507 U/L (n.v. 38-174);

- Urine test: within normal limits;

- Encephalic and medullary MRI with contrast medium (03.09): The structures on the median line are regular. No foci alterations of the signal intensity under and above the tentorium. No recent lesions spreading out. No pathological enhancements after gadolinium; in particular, regular acoustic-facial package bilaterally. Rachis in axis. Regular morphology and intensity of the signal in the analyzed marrow. No pathological enhancements after gadolinium. Unco-vertebral-arthrosis C5-C6 with intersomatic disk, with consistent protrusion onto the osteodegenerative alterations. Dorsal disks in the inferior-medium tract show reduced thickness and degeneration. First-degree anterior spondylolisthesis of L3 over L4 with minor protrusion of the intersomatic disk. The L4-L5 and S1 show reduced thickness, degeneration, and minor protusion. Conus at place. Regular cauda.

- Lower abdomen ultrasound scan (03.09): Relaxed bladder with 220 cc capacity, with no parietal endoluminal alterations. No residuals after miction.

- Urofluxometry: within normal limits; During the hospitalization, the therapy was well tolerated with no relevant side effects.

For the next days, we suggest abundant hydration. The rehabilitation therapy can be usefully maintained. The patient will contact us in 2 months for a possible new therapy cycle”.

The patient reports that the disease progress lead to a relevant recovery during the first year. Nevertheless, during the following years the recovery was limited and, currently, the disease progress is stable, with possible minor improvements.

Expert's Opinion

It seems to me that the most likely diagnosis in this case is acute myeloradiculoneuritis. The initial symptoms of acute asymmetric paraperesis, sensory level and electric shock sensation in both lower extremities are typical of myelitis. Myelitis was also demonstrated in the MRI. The root enhancement and EMG findings indicate also peripheral nervous system (PNS) involvement. The lack of pyramidal signs may be due to the PNS involvement or involvement of the anterior horns at the distal marrow segments.

Regarding the questions raised by the patient:

1. It seems to me that the diagnosis is acute myeloradiculoneuritis. The marrow (spinal cord) involvement is beyond the accepted symptoms for GBS. Typically, GBS affects the roots and the peripheral nerves without myelitis. In the patient's case there was clearly myelitis. Usually, this is a monophasic illness and it is thought to result from infectious disease (usually viral) or immune-mediated process, though often no viral agent is detected. I absolutely agree with the treatment given to the patient.

2. At that stage I think that there is no specific neurologic therapy. It seems that the patient has remained with a permanent neurologic sequelae of the acute insult, and that the disease is not expected to progress in the future. Therefore, I doubt if there is any benefit in continuing IVIG and steroid therapy. I think that the only therapy is rehabilitation and the use of supportive instrumenation to the weak left leg.

3. I think that at this stage, more than 4 years after onset of symptoms, the likelihood of neurologic recovery or improvement is very low.

4. Since the right leg is strong, supportive instrumentation to the left leg may be helpful and may replace the use of crutches. I therefore suggest consultation with physical rehabilitation specialist.

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