Case Information
•Patient: Male, 52 years old, admitted on 2024-07-07.
•Chief complaint: Difficulty swallowing with choking on liquids for 18 hours.
•Present illness: The patient noticed difficulty swallowing and choking on liquids 18 hours ago (2024-07-06 15:00) after waking from a nap (no abnormalities were noted at 13:00 when the nap began), accompanied by slurred speech, no significant limb numbness or weakness, no visual disturbances such as blurriness, diplopia, or blackout. These symptoms persisted without relief, leading to an emergency visit where the examination revealed: speech disorder, full ocular movement in both eyes, symmetrical forehead and nasolabial folds, bilateral limb muscle strength at grade V, NIHSS score: 1 point. The patient was treated with intravenous alteplase, but symptoms showed no significant change.
•Past medical history: Hypertension diagnosed 1 month ago, with a maximum reading of 150/100 mmHg, currently taking “Benidipine,” with no regular blood pressure monitoring. Smoking history of over 30 years, averaging 20 cigarettes per day; occasional alcohol consumption.
•Family history: No significant findings.
•Admission examination: Blood pressure 120/80 mmHg, alert, speech disorder, cranial nerve examination (-), bilateral limb muscle strength at grade V, finger-nose test and heel-knee-shin test stable and accurate, sensory examination showed no significant abnormalities, bilateral tendon reflexes symmetrical (+), no pathological signs elicited. Water swallowing test: grade 5.
•Auxiliary examinations: Cardiac ultrasound, head CT + CTA + CTP showed no significant abnormalities.
•2024-07-08
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Symptoms worsened, presenting as weakness in all four limbs, particularly in the upper limbs, with difficulty lifting and gripping in both upper limbs, while both lower limbs could be lifted. Difficulty swallowing and choking on liquids showed no significant change.
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History inquiry: The patient reported having fever and similar cold symptoms about 2 weeks prior.
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Examination: Alert, speech disorder, full ocular movement in both eyes, no nystagmus or diplopia. Symmetrical forehead and nasolabial folds, weak eyelid closure. Tongue protrusion centered. Proximal muscle strength in both upper limbs at grade II, distal muscle strength at grade III, proximal muscle strength in both lower limbs at grade IV. Other findings were the same as before.
•Considered diagnosis: GBS, lumbar puncture to be completed, and immunoglobulin therapy initiated at 30g qd + Methylprednisolone 1g qd.
•2024-07-09
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Weakness in all four limbs worsened, with inability to lift shoulders in both upper limbs, weak grip, and inability to lift both lower limbs.
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Examination: Alert, speech disorder, weak eyelid closure. Proximal muscle strength in both upper limbs at grade 0, distal at grade II+, proximal muscle strength in both lower limbs at grade III, distal at grade II. Sensory examination showed basic symmetry in pinprick sensation. Bilateral tendon reflexes diminished. No pathological signs. Other findings were the same as before.
•2024-07-10
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Symptoms continued to worsen, with weak respiratory effort and inability to lift all four limbs.
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Examination: Alert, speech disorder, incomplete eyelid closure. Proximal muscle strength in both upper limbs at grade 0, distal at grade 1, muscle strength in both lower limbs at grade 1. Sensory examination showed basic symmetry in pinprick sensation. Bilateral tendon reflexes diminished. No pathological signs. Other findings were the same as before.
•2024-07-12
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The patient showed slight improvement in limb weakness and respiratory effort.
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Examination: Alert, speech disorder, incomplete eyelid closure. Muscle strength in both upper limbs at grade 1, muscle strength in both lower limbs at grade 1+. Sensory examination showed basic symmetry in pinprick sensation. Bilateral tendon reflexes diminished. No pathological signs.
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Laboratory results returned: Cerebrospinal fluid cell count within normal range, cerebrospinal fluid protein: 0.65g/L, cerebrospinal fluid and blood GM1-IgG and other ganglioside antibodies, NMDA-R-Ab and AQP-4-Ab were all negative, cerebrospinal fluid TORCH negative.
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Electromyography: Slowed motor nerve conduction velocity in both ulnar and common peroneal nerves; slowed sensory nerve conduction velocity in both ulnar, median, and sural nerves.
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Head MR and cervical spine MR showed no relevant abnormal lesions.
• As of 2024-07-23
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The right side could move across the bed but could not lift off the bed, the left side was slightly worse, and both lower limbs could slide on the bed.
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Examination: Alert, speech disorder, incomplete eyelid closure. Proximal muscle strength in the right upper limb at grade II, distal at grade III, proximal muscle strength in the left upper limb at grade I, distal at grade II, proximal muscle strength in both lower limbs at grade II+, distal at grade III, other findings were the same as before.
• Discharged to a rehabilitation hospital for continued rehabilitation treatment.
Separation
Line
Oropharyngeal-Cervical-Brachial Variant of GBS
CASE 1
•Male, 57 years old.
•Admitted due to “speech disorder and generalized weakness for about 9 hours.”
•The patient noticed slurred speech, weakness in all limbs (especially in both upper limbs), unsteady walking, accompanied by numbness in all limbs (mainly in both upper limbs), abdominal distension, hoarseness, and a sensation of a foreign body in the throat, with difficulty expectorating.
•Visited the emergency department, where the stroke green channel doctor did not rule out brainstem stroke, and an emergency cranial MR was performed, showing no abnormalities.
•Blood routine and biochemical tests indicated potassium level at 2.96 mmol/L, and potassium supplementation was given for hypokalemia, with plans for enhanced MRI of the cervical, thoracic, and lumbar spine the next day.
•The next morning, the patient’s condition worsened, with new onset of swallowing difficulties, ptosis, slow eating, and weak chewing, along with chest tightness.
•History inquiry: The patient reported having upper respiratory infection symptoms 10 days prior to admission.
•Neurological examination:Speech disorder, nasal voice, mild ptosis in both upper eyelids, full ocular movement, pharyngeal congestion, uvula deviated to the right, significantly reduced pharyngeal reflex, slight neck resistance, normal muscle tone, proximal muscle strength in both upper limbs at grade IV, distal muscle strength at grade V, tendon reflexes diminished, symmetrical reduction in deep and superficial sensation, negative pathological signs.
•Laboratory tests: Blood routine, liver and kidney function, blood gas analysis were all normal, and electrolyte recheck showed potassium ion at 4.1 mmol/L.
•Cranial MR and enhanced cervical and thoracic spine MR showed no abnormal signals.
•Chest CT: Multiple infectious lesions in the right middle lobe, left upper lobe, and both lower lobes.
•That afternoon, a lumbar puncture was performed, cerebrospinal fluid pressure was normal, and routine and biochemical tests showed no abnormalities. Neurophysiological tests showed no abnormalities.
•Serum:Anti-GT1a antibody IgG (++), anti-GT1b antibody IgG (+), anti-GQ1b antibody IgG (++)..
•Cerebrospinal fluid peripheral nerve-related antibodies were all negative.
•A full course of IVIg (0.4g/kg daily for 5 days) was administered, and the day after treatment, the patient’s condition stabilized and did not worsen.
•One week later, the patient could walk normally, symptoms of limb numbness disappeared, and two weeks later, only mild speech slurring remained upon discharge. A follow-up call one month later indicated the patient had basically returned to normal.
CASE 2
•Female, 60 years old.
•Admitted on February 27, 2021, due to “dizziness for 3 days, worsening with slurred speech for 1 day.”
•Three days prior, the patient experienced dizziness without obvious cause, accompanied by rotational visual disturbances, blurriness, and nausea/vomiting. After treatment for “vertigo” at another hospital, dizziness worsened, and slurred speech and limb weakness developed, leading to hospitalization.
•Upon admission: Alert, mild speech disorder, full ocular movement in both eyes, symmetrical nasolabial folds, tongue protrusion centered, muscle strength in both upper limbs at grade V-, normal muscle strength in both lower limbs, normal tendon reflexes, no pathological signs, normal sensory coordination, water swallowing test at grade I.
•On the second day after admission, the patient’s symptoms continued to worsen, with the onset of swallowing difficulties. Examination revealed: weakness in raising the right soft palate, absent pharyngeal reflex, proximal muscle strength in both upper limbs at grade III, water swallowing test at grade IV, and the patient was given a nasogastric feeding of liquid diet. A multi-modal MR assessment showed no abnormalities.
•Subsequently, the patient’s symptoms progressed, with diplopia, difficulty expectorating, inability to lift the head, and weakness in lifting both upper limbs.
•On the fifth day of hospitalization, examination revealed: severe speech disorder, fixed ocular position, inability to extend the tongue, neck flexor and extensor muscle strength at grade III, proximal muscle strength in both upper limbs at grade I, distal muscle strength at grade IV, normal muscle strength in both lower limbs, normal tendon reflexes, normal sensation, positive Babinski sign bilaterally.
•Cranial MR showed no intracranial lesions.
•EMG: High-frequency repetitive stimulation showed no abnormalities; left ulnar and median nerve Erb’s point stimulation compound muscle action potential (cMAP) amplitude decreased, bilateral musculocutaneous and axillary nerves cMAP amplitude decreased, and bilateral axillary nerve motor conduction velocity slowed, indicating multiple peripheral nerve damage (more pronounced proximally).
•Serum antibody testing: Anti-GT1a antibody IgG (++) and anti-GQ1b antibody IgG (++)..
CSF testing: Normal pressure, normal cell count, positive Pandy’s test, protein level at 624.10 mg/L, CSF IgG at 18.8 g/L.
•IVIg therapy was administered intravenously at a dose of 0.4g/(kg·d) for 6 days. The patient’s symptoms gradually improved, and on the sixth day of treatment, the patient’s speech disorder and soft palate elevation improved compared to before, with partial abduction of both eyes, and proximal muscle strength in both upper limbs at grade IV.
CASE 3
•Female, 57 years old.
•Admitted with “slurred speech for 8 days, worsening with weakness in both upper limbs and neck for 6 days, and weakness in both lower limbs for 2 days.”
•Eight days prior, the patient experienced slurred speech, choking on liquids, and swallowing difficulties. Six days prior, the patient experienced weakness in lifting the neck and difficulty lifting both upper limbs, and two days prior, weakness in both lower limbs developed, with chest tightness and shortness of breath one day prior. There were no visual disturbances, sensory abnormalities, urinary or bowel dysfunction, or consciousness disturbances during the course of the illness.
•Neurological examination: Poor movement of both soft palates, inability to lift the neck, proximal muscle strength in both upper limbs at grade II, distal muscle strength at grade III+, proximal muscle strength in both lower limbs at grade IV+, bilateral tendon reflexes (+), negative pathological signs, and no abnormalities in other cranial nerves, sensation, or coordination.
•Supplementary past history: One month prior to the illness, the patient was diagnosed with “subacute thyroiditis and upper respiratory infection” at a tertiary hospital due to fever, cough, and neck discomfort, with no history of diarrhea.
•Cranial and cervical spine MRI showed no significant abnormalities, and electrophysiological examination indicated decreased amplitude of compound muscle action potentials in bilateral median, ulnar, and axillary nerves, with prolonged distal latency in bilateral tibial nerves and decreased amplitude.
•Serum ganglioside antibodies (GQ1b, GT1b, GD1a, GD1b, GM1, GM2, GM3) IgG and IgM were all negative.
•IVIg was administered at a dose of 0.4g/kg·d, and on the 10th day, the patient developed eyelid weakness, drooling, and bilateral peripheral facial paralysis, with improvement in neck and both upper limb muscle strength on the 11th day.
•On the 18th day of illness, the patient could drink without choking, could lift the neck, proximal muscle strength in the left upper limb at grade III, proximal muscle strength in the right upper limb at grade IV, distal muscle strength at grade IV+, and no improvement in peripheral facial paralysis upon discharge.
CASE 4
•Male, 69 years old.
•Admitted with “slurred speech and swallowing difficulties for 7 days.”
•Seven days prior, the patient experienced slurred speech without obvious cause, and six days prior, developed incomplete closure of the right eyelid and left-sided mouth droop. Five days prior, the patient experienced difficulty lifting both upper limbs, difficulty squatting and standing, could walk on flat ground, choking on liquids, swallowing difficulties, difficulty lifting the neck, accompanied by coughing and expectoration, with no sensory abnormalities or consciousness disturbances during the course of the illness. The patient had a nasogastric tube placed at a local hospital, cranial MRI showed no abnormalities, and lumbar puncture cerebrospinal fluid showed IgG at 50mg/L (slightly elevated), with routine and biochemical tests within normal range.
•Admission examination: Right peripheral facial paralysis, full ocular movement in all directions, speech disorder, poor movement of both soft palates, inability to lift the neck, proximal muscle strength in all limbs at grade III, distal muscle strength at grade V, normal sensory examination, normal coordination, negative pathological signs.
•IVIg was administered at a dose of 0.4g/kg·d for 5 days. On the fifth day of treatment, the patient removed the gastric tube, could eat independently, and neck strength improved.
•On the 17th day of illness, lumbar puncture indicated: white blood cell count 8×10^6/L, protein 554mg/L; serum ganglioside antibodies (GQ1b, GT1b, GD1a, GD1b, GM1, GM2, GM3) IgG and IgM were all negative. On the 18th day, the patient could lift the neck normally, swallowing showed no significant abnormalities, proximal muscle strength in all limbs at grade IV-, distal muscle strength at grade V upon discharge.
Summary of the characteristics of these 4 cases:
1.Bulbar paralysis symptoms appeared early and were prominent.
2.Weakness in the neck and shoulder region.
3.Weakness in both upper limbs or weakness in all limbs with a predominance in both upper limbs.
4.Cerebrospinal fluid protein-cell dissociation, some antibodies positive, cMAP amplitude decreased.
5.Good effect of immunoglobulin therapy.
Oropharyngeal-Cervical-Brachial Variant of GBS
•In 1986, Ropper first described three patients with rapidly progressive weakness of the oropharyngeal, cervical, and upper limb muscles, which he termed the PCB variant of GBS, considering it a special type of GBS.
•Nagashima et al. showed that 68% of patients with oropharyngeal-cervical-brachial GBS presented with lower limb symptoms, 60% had sensory abnormalities, and other symptoms included ocular muscle paralysis (55%), ataxia (43%), with proximal weakness in the upper limbs accounting for 47%, distal weakness for 28%, and severe weakness in the lower limbs (muscle strength ≤ grade III) for 31%. It is also reported that some patients may only present with neck and brachial plexus symptoms, or only oropharyngeal symptoms without other cranial nerve and limb symptoms.
•The immune mechanism of GBS is believed to involve self-antibodies binding to gangliosides, activating the complement system, forming membrane attack complexes, leading to nerve conduction block and ultimately secondary axonal degeneration, which is the main pathological feature of axonal GBS.
•In 60% of GBS patients, acute phase serum can detect anti-ganglioside antibodies, and different subtypes of GBS have different types of ganglioside-specific antibodies.
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GQ1b→Strongly expressed in oculomotor, trochlear, abducens nerves, deep cerebellar nuclei, brainstem reticular structures, and muscle spindles → symptoms of ocular muscle paralysis, ataxia, consciousness disturbances, etc.
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GT1a→Neurons in the oropharyngeal and cervical muscles, with expression in the glossopharyngeal and vagus nerves significantly stronger than GQ1b, and similar expression in ocular motor nerves as GQ1b → weakness in oropharyngeal and cervical muscles.
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Anti-GT1a antibodies are specific antibodies for the PCB variant, while anti-GQ1b antibodies are specific antibodies for MFS and Bickerstaff encephalitis, with cross-reactivity between the two.
Characteristics
•The age of onset for PCB variant GBS ranges from 5 to 83 years, with an average age of 43, male to female ratio of 1.3:1.
•Preceding infections are very common in patients with PCB variant GBS, with respiratory infections being the most common.
•Core symptoms: Rapidly progressive oropharyngeal, cervical, and brachial weakness, accompanied by diminished or absent upper limb tendon reflexes, which may overlap with other variants.
•50% of patients with PCB variant GBS have normal cerebrospinal fluid protein levels within the first week of onset.
•Early cerebrospinal fluid and neurophysiological examinations may be normal in PCB variant GBS, thus a diagnosis of PCB variant GBS cannot be ruled out even if no abnormalities are found in neurophysiological examinations early in the disease; it is recommended to recheck after several days.
Diagnostic criteria
•If the following symptoms appear, one should be alert to the possibility of PCB variant GBS:
① Symmetrical weakness of oropharyngeal, cervical, and upper limb muscles;
② Diminished or absent tendon reflexes in both upper limbs;
③ Monophasic course, with a time interval of 12h to 28d from onset to peak weakness.
•Additionally, the following symptoms highly support the diagnosis of PCB variant GBS:
① Preceding events (such as upper respiratory infection, diarrhea, trauma, etc.);
② Cerebrospinal fluid protein-cell dissociation (cell count <50/μl, protein >0.45g/L);
③ Neurophysiological evidence, such as decreased cMAP amplitude, absence of F waves, conduction block, etc.;
④ Positive anti-GT1a or anti-GQ1b antibodies.
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