Onset with "bitemporal pain", don't just think of Guillain-Barre syndrome

*For medical professional reading reference only

Without further ado, just look at the case!

An 18-year-old female with obesity presented to the emergency department of our hospital mainly because of "sudden onset of severe bitemporal pain for 2 days".

Physical examination:

• He was clearly conscious and had no light perception in both eyes.
  
  
  
  

  Past history of rheumatic heart disease

  
  
  
  

  Auxiliary inspection:

  
  
  • Echocardiography showed mitral valve thickening, mitral regurgitation, and aortic regurgitation
    
    

    Combined with the patient's medical history, clinical manifestations and auxiliary examinations, what is the disease of the patient, is it Guillain-Barre syndrome (GBS)?

    
    
    
    
    
    
    
    

    However, first in most reports of "GBS with papilledema", the characteristics of the case where the disease progressed to elevated intracranial pressure with limb weakness were different from ours

    
    
    
    

    Second, blindness as a complication of "GBS with papilledema" has not been reported in the past
    .
    
    

    Third, nearly all previously reported cases of "GBS with papilledema" that I have seen have elevated CSF proteins (with one exception)
    .
    
    

    Moreover, in this case, peripheral nerve conduction velocities were normal, so the diagnosis of GBS is also not supported
    .
    
    

    In addition, the patient's elevated intracranial pressure made the author think about idiopathic intracranial hypertension, and regarded it as a provisional diagnosis, so the patient underwent a lumbar-peritoneal shunt and was given acetazolamide 250 mg 3/day orally
    .
    
    

    After 5 days, the patient's papilledema improved
    .
    
    After 2 weeks, the patient's visual acuity improved, manual movement was visible in the left eye, light perception was visible in the right eye, and the pupillary paralysis also recovered, the limb weakness also recovered, and the patient could walk with support
    .
    

    In this case, the patient had acute onset, bilateral vision loss, ophthalmoplegia, flaccid paralysis, and normal feeling with elevated intracranial pressure
    .
    
    Head MRI+MRV+cervical spine MR showed no abnormalities related to these symptoms
    .
    
    The reduction in intracranial pressure resulted in a marked improvement in clinical symptoms, suggesting a causal relationship between intracranial pressure and these manifestations
    .
    
    While blindness and ophthalmoplegia are well-known clinical manifestations of idiopathic intracranial hypertension, flaccid paralysis is a rare presentation that can interfere with rapid diagnosis and treatment
    .
    
    

    
    Definition of idiopathic intracranial hypertension
    

    Idiopathic intracranial hypertension is a clinical syndrome characterized by elevated intracranial pressure in the absence of intracranial space-occupying lesions or imaging and without dural sinus thrombosis
    .
    
    

    
    Clinical manifestations of idiopathic intracranial hypertension

    • Headache, total cranial headache, worse upon awakening and may be aggravated by movements that raise intracranial pressure;

    • Papilloedema and vision loss: It usually occurs symmetrically, but can be unilateral
      .
      
      Papilloedema can be accompanied by peripheral hemorrhage, exudate, and macular edema
      .
      

    • Tinnitus is a common symptom of idiopathic intracranial hypertension and can be unilateral or bilateral, intermittent or continuous
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      Symptoms are worse at night
      .
      

    • Other symptoms: diplopia, facial paralysis, and hearing loss
      .
      
      

    
    The pathogenesis of idiopathic intracranial hypertension

    As the name suggests, the etiology of idiopathic increased intracranial pressure is unknown, and the most likely mechanism to explain radiculopathy in patients with idiopathic intracranial hypertension is that the increased intracranial pressure dilates the subarachnoid space and mechanically compresses the nerve roots
    .
    
    Regarding the etiology of idiopathic intracranial hypertension, there are several theories: (1) impaired cerebrospinal fluid balance (decreased reabsorption or excessive production of cerebrospinal fluid); (2) altered vitamin A metabolism, related to tetracycline and steroid drugs; (3) venous sinus stenosis , which can lead to increased venous pressure, decreased cerebrospinal fluid absorption, increased intracranial pressure, and venous sinus hypertension
    .
    

    Regarding the mechanism of increased intracranial pressure in idiopathic intracranial hypertension, it must be stated that obese young women are more likely to obtain it.
    Theories include increased cerebral water content, excessive cerebrospinal fluid production, decreased cerebrospinal fluid absorption, and increased cerebral venous pressure
    .
    
    Some people have put forward the theory of endocrine disorders, mainly related to the active secretion of sex hormones and adipose tissue
    .
    

    The role of cerebral venous hypertension in the pathogenesis of idiopathic intracranial hypertension is still controversial.
    Earlier studies have shown that intracranial pressure is directly caused by increased intra-abdominal pressure, through diaphragm elevation, pleural pressure Elevated, decreased cerebral venous return, thus persistent cerebral venous hypertension
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    However, this theory requires central obesity rather than extremity obesity to raise intra-abdominal pressure, and does not explain why women are susceptible
    .
    
    The current trend is more about the role of transverse venous sinus stenosis in the pathogenesis of idiopathic intracranial hypertension
    .
    

    
    Diagnostic criteria for idiopathic intracranial hypertension

    1.
    If there are symptoms, it may only reflect intracranial hypertension or papilledema;

    2.
    If there are signs, it may only reflect intracranial hypertension or papilledema;

    3.
    Confirmation of increased intracranial pressure (lateral position);

    4.
    The CSF composition is normal;

    5.
    For typical patients, MRI or enhanced CT without hydrocephalus, mass, structure or vascular lesions; for other (atypical patients), MRI and MRV are required;

    6.
    No other reasons related to increased intracranial pressure
    .
    
    

    
    treat
    

    ▌ Medication

    Carbonatase inhibitors, such as acetazolamide, are the mainstay of treatment for idiopathic intracranial hypertension, and topiramate is the most commonly used in the treatment of headaches because it is also a weak carbonatase inhibitor.
    Effective in patients with primary headache with papilledema
    .
    
    

    ▌ Surgery

    Optic nerve sheath fenestration is often recommended for those with severe vision loss but mild or no headache, and cerebrospinal fluid shunting, such as a ventriculo-peritoneal shunt, is often recommended for some patients with vision loss, papilledema, and headache surgery or lumbar-peritoneal shunt
    .
    
    

    
    prognosis
    

    The prognosis for most patients with idiopathic intracranial hypertension is favorable
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    However, many patients have persistent headaches leading to decreased quality of life and even the occurrence of depression
    .
    

    When idiopathic intracranial hypertension is associated with identified triggers, such as anemia, untreated obstructive sleep apnea, interruption of medication, and other factors often lead to rapid disease progression
    .
    
    The main symptom of idiopathic intracranial hypertension is vision loss due to papilledema.
    Most patients experience visual impairment, and up to 31% of patients will experience irreversible vision loss
    .
    

    However, a small proportion of patients with idiopathic intracranial hypertension have a more acute onset, with rapid onset of signs and symptoms of increased intracranial pressure, and rapid deterioration of vision loss within a few days.
    In the study of cases of "severe intracranial hypertension", all patients lost vision, and after surgical treatment, 50% of patients remained blind in both eyes
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    Therefore, timely identification and early aggressive treatment of patients with idiopathic intracranial hypertension are crucial
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    References: [1] Li Ke, Wang Feng.
    Advances in diagnosis and treatment of idiopathic intracranial hypertension and related eye diseases.
    Chinese Journal of Interventional Radiology.
    2020, 8(03).
    [2] Tian Guohong.
    Over time – 1 case Diagnosis and treatment of patients with idiopathic intracranial hypertension.
    Chinese Journal of Ophthalmology and Otolaryngology.
    2019, 19(03).