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Cranial Nerve Surgery Disease:Nerve vascular compression syndrome

There are Facial Spasm, Trifacial neuralgia, Glossopharyngeal neuralgia, Spastic torticollis, and Oculomotor paralysis in nerve vascular compression syndrome. These are nervous symptoms developed by which cerebral nerve is compressed by adjacent vessels in endocranial. Symptoms are disappeared in releasing compression of blood vessel to nerve by surgical operation (microvascular decompression).

Variation of nerve vascular compression syndrome
Disease Symptom nerve vascular
Facial Spasm Twitch on one side of face Facial nerve (7th)
Trifacial neuralgia Pain on face Trifacial nerve (5th)
Glossopharyngeal neuralgia Residual feeling of fish bone on throat Glossopharyngeal nerve (9th)
Bout of vertigo Buzzing is accompanied with dizziness Vestibular cochlear nerve (8th)
Spastic torticollis Leaning inclination of the face Accessory nerve (11th)
Lingual atrophy Lingual atrophy on one side Hypoglossal nerve (12th)
Oculomotor paralysis Sagging eyelid Optical nerve (2nd)
Half-body perception disorder> Sensation abnormality on half-body Compression of vertebral artery in medulla
High-blood pressure High-blood pressure Compression of the bridge medulla by vertebral artery

Facial Spasm – one side is a therapeutic objective

The symptom of one side facial spasm becomes the therapeutic objective. There is also the essential facial spasm (Meige syndrome) exists but this Meige syndrome is not the objective of facial nerve surgery (microvascular decompression)

The cause of one side facial spasm is at all by blood vessel compression at the exit of the root of facial nerve in brain-stem region. But other than this cause, there are, the cause by developed after facial nerve paralysis (damage on facial nerve: Bell paralysis), the cause, in addition to arachnoid adhesion after surgery, after brain-stem infarction or epileptic one, or the one which same side cerebellar cavernous hemangioma or brain tumor compresses the blood vessel then the blood vessel compresses facial nerve. They need to be differentiated.

Convulsiveness on inferior eyelid is a beginning.

Observation of left facial nerve course is deviated by blood vessel. One of component is artificial synaptic conduction theory by facial nerve by Nielsen, and the other is Jannetta theory in which facial nerve transit from central myelin to peripheral myelin at few mm to 1.0cm away section from root exit zone of brain-stem. Central myelin is formed with oligodendroglia (central nerve region), and peripheral myelin is formed with schwann cell (peripheral nerve). In case neighborhood of this is compressed by blood vessel, denaturalization or demyelination is produced, inter-axon transmission is raised, and excitability of facial nerve core is increased, then facial spasm is occurred. Damaged part of nerve works as evoking region, abnormal impact occur, evokes facial nerve core activity, then send impulse signal to develop spasm. Symptoms are to begin with lower eyelid spasm (twitching), repeating remission and hatred, and finally spasm is produced all the time. Its characteristics are; to be set up with mental strain especially when meeting with someone. It is easy to be diagnosed by means of where facial spasm is produced with blink test at rest. With the degree of symptom, there are, grade I: contraction from Orbicularis Oculi Muscle surroundings to upper face, grade II: entire face spasm reached to lower jaw, and grade III: the one reached to platysma muscle on anterior neck.

Curing rate by surgery is 90%.

Treatment is divided mainly in surgery and Botox injection. There is almost no effect in oral dosage therapy with anticonvulsant agent. Surgery is neuro-vascular decompression surgery, and it is called as micro vascular decompression surgery, which Gardner had clarified with its component in 1962 and Jannetta actually made a report for this treatment method in 1966. In Japan, it has begun using this surgical method as established treatment in 1980s. Cure rate by surgery is about 90% now.

There is a big difference on developmental rate for complicating disease by surgical operator.

Complicated disease is, although there are some differences in each report from other entities, seen in 3% to 24% of the cases, and its main symptoms are hearing lost/reduction, facial nerve paralysis, cerebellar hemorrhage, cerebrospinal fluid leakage, brain-stem infarction, and cerebral meningitis.
As for the writer’s case, 472 cases are experienced by 2010, and 460 of them are cured completely (97.5%), and 2 cases out of remaining 12 cases, it has cured by re-operative surgery. Regarding complicated disease, permanent complication disease (2.8%) has appeared with; hearing lost occurred in 8 cases, hearing reduction occurred in 2 cases, facial nerve paralysis in 1 case, cerebrospinal fluid leakage in 1 case, and brain-stem infarction in 1 case. And transient delayed facial nerve paralysis (one month later from surgery) has occurred in 2 cases.
Surgical method is; first about 6cm long skin incision is made at 5cm back from ear attached area, exposes the occipital bone, then craniotomy is performed. Bone powder or bone fragments picked up through the operation are used at bone formation later. Expose facial nerve and hearing nerve a compress cerebral brain just a little bit, then cut open arachnoid mater enough at the rear of glossopharyngeal and vagus nerve and expose the exit portion of facial nerve. Then fix the compressed blood vessel and move it to prevent nerve from receiving blood vessel compression. For the move, in case it is thick vertebral artery, hang it with silk thread to upper dura mater, or wrap around compressing blood vessel by Gore-Tex blood vessel for blood vessel beating does not affect to nerves. Bone formation at the open section is first making artificial bone fragment in the size of lost section with using fibrin glue, bone powder and bone fragments, then engage it and apply fibrin glue to fix. Surgery is completed with suture the skin.

Trifacial neuralgia – symptom is strong pain on innervation area.

三叉神経の支配領域 Complicated disease is, although there are some differences in each report from other entities, seen in 3% to 24% of the cases, and its main symptoms are hearing lost/reduction, facial nerve paralysis, cerebellar hemorrhage, cerebrospinal fluid leakage, brain-stem infarction, and cerebral meningitis.
As for the writer’s case, 472 cases are experienced by 2010, and 460 of them are cured completely (97.5%), and 2 cases out of remaining 12 cases, it has cured by re-operative surgery. Regarding complicated disease, permanent complication disease (2.8%) has appeared with; hearing lost occurred in 8 cases, hearing reduction occurred in 2 cases, facial nerve paralysis in 1 case, cerebrospinal fluid leakage in 1 case, and brain-stem infarction in 1 case. And transient delayed facial nerve paralysis (one month later from surgery) has occurred in 2 cases.
Surgical method is; first about 6cm long skin incision is made at 5cm back from ear attached area, exposes the occipital bone, then craniotomy is performed. Bone powder or bone fragments picked up through the operation are used at bone formation later. Expose facial nerve and hearing nerve a compress cerebral brain just a little bit, then cut open arachnoid mater enough at the rear of glossopharyngeal and vagus nerve and expose the exit portion of facial nerve. Then fix the compressed blood vessel and move it to prevent nerve from receiving blood vessel compression. For the move, in case it is thick vertebral artery, hang it with silk thread to upper dura mater, or wrap around compressing blood vessel by Gore-Tex blood vessel for blood vessel beating does not affect to nerves. Bone formation at the open section is first making artificial bone fragment in the size of lost section with using fibrin glue, bone powder and bone fragments, then engage it and apply fibrin glue to fix. Surgery is completed with suture the skin.

左三叉神経が血管にて圧迫されている(矢印) The symptom is paroxysmal strong pain and its developed region is commonly at II or III branch field. It is developed mainly among over 40 years older people. Nerve pain itself does not last long, about few seconds to several tens of seconds. It may be triggered with drinking cold drinks, washing face, brushing of teeth, shaving, eating etc., and pains are getting stronger if pain area is touched. There is pain attack induced area so that if in case this area gets irritated, paroxysmal pain is induced. It is important to distinguish from sphenopalatine neuralgia.

Surgical operation is the only radical treatment.

In oral administration treatment, Carbamazepine, Baclofen, Phenytoin, Clonazepam, Chinese herbal medicine (Keishikajutsubuto, Saikokeishito, syosaikoto) are used, but Carbamazepine is used in common. Surgical treatment (Neuro-Vascular Decompression Surgery) is the only radical treatment and its cure rate is said in general 70% to 80%. There is a rare case where petrosal vein causes this disease, thus writer has experienced this vein pass through trifacial nerve. According to the self-experiment cases at writer’s hospital, 145 cases (92.4%) among 157 cases is completely cured by micro-vascular nerve decompression surgery.
Accession to trifacial nerve region has no problem at outside of cerebellum, but occasionally, it has to select the access method along the way of superior part of the cerebellum to cerebellar tentorium, unlike facial spasm, craniotomy surgery needs to be done in such the way that craniotomy upper edge and outer edge are to be placed in the angle of lateral sinus and S-shaped transverse sinus. As same as craniotomy, craniotomy with rough 2.5cm open cut is performed and fix the compressing blood vessel, move it so that no compression has exert influence. There is a case where petrosal vein may disturb the sight, but although try to preserve but can be cut one out of few by cauterization without further problem. However, if in case it is thick nerve case, the cut by cauterization may cause Venus infarction and advanced to cerebellar hemorrhage. As compressed blood vessel exists at the back side of trifacial nerve, it sometimes looks as no compressing blood vessel. It is necessary to pay attention with. It is necessary to move it completely further away than the one at facial spasm.

Other trifacial neuralgia treatment

There are below listed treatment method for trifacial neuralgia.

Gamma Knife

Trifacial Neuralgia treatment by Gamma Knife is left the compressing vessel as is and irradiate radioactive ray to trifacial nerve so that the feeling against the pain is changed. Surgical treatment can relieve pain right after the surgery, but it may take 3 to 4 months before having actual effects from this method. Cure rate where no drug administration is necessary, is about 60%. This is the appropriate method for the patients who has difficulty for surgery, but cure rate is not high enough like surgical treatment. Adverse effect is facial numbness and develop the sense of discomfort, and they are appeared in about half-year to 2 years after surgery.

Nerve Block

This method is to block ganglion gasseri from oral cavity, but dis-advantage is that only proficient doctor can perform this.

Glossopharyngeal neuralgia – rare disease in nerve vascular compression syndrome.

This disease is a rare case in nerve vascular compression syndrome, and it often expressed commonly as “feel pain as caught fish bone in the throat” when swallow something. 4 cases out of experienced 5 cases has completely cured. Development frequency becomes lower in comparison with facial spasm or trifacial neuralgia.

Spastic torticollis – It is important to identify the difference with oblique neck by drugs.

In spastic torticollis, there are not so many cases applicable for surgical operation. It is important to distinguish the difference with oblique neck by drug or mental. Treatment is Botox injection and nerve decompression.

Dizziness – diagnose from compression on auditory nerve.

There are many and boarder area causes for clinical dizziness so that description about this disease may take bunch of papers. Here instead, dizziness produced by vestibular nerve compression by blood vessel is explained. It is produced by #8 nerve compression.

Symptoms

Rotary dizziness for longer time, buzzing and auditory disturbance

Diagnosis

Patient reaction lowered at caloric test
Confirmation of auditory nerve compression with MRI. Prolong the I-III Waves Latency in brainstem auditory evoked potentials.

Differentiation
Otitic
Other than otitis (Visual device, cervical spine, nasal pharyngeal, central nervous system disease)
Combination with entire body disease (anemia, polycythemia, diabetes, hypotension, high-blood pressure, arrhythmia)
Psychogenesis, autonomic nerve system, epileptic

Treatment method is surgical operation, but the indication of surgery becomes the subject for the patient who has rotary dizziness, buzzing and auditory disturbance for longer period and correspond to the above-mentioned diagnosis. Dizziness complicated with facial spasm is produced by same mechanism so that such case is omitted here. Surgery is performed for 6 cases, and 4 cases are completely cured, dizziness degree and frequency has been apparently reduced and becomes the degree of mild spasm appears merely in 2 cases.

Oculomotor paralysis - Eye movement disorder or pupil inequality occur.

Regarding the peripheral motion ocular nerve paralysis, aneurysm which makes projection to outside of bifurcation area of internal carotid artery and posterior communicating artery, and upper cerebellar aneurysm compresses motion ocular nerve then produces neuropathy on motion ocular nerve so that blepharitis drooping eyelids, ocular motility disorder and anisocoria are produced, are in common.

Here, the case, where nerve is compressed by blood vessel deviated by arterial sclerosis, are the objectives. In experienced 14 cases, all cases are completely cured including a case where disease has been produced on both eyelids with the lag period (Neuro Med Chir (Tokyo) 31: 45-48, 1991). Nerve compression release, which is occurred by vessel movement, is possible by pterional approach, and occasionally subtemporal approach becomes necessary. Time from the appearance of symptom to surgery is the longest in 1.5 months, and unlike oculomotor paralysis caused by the compression from aneurism, there is an impression for the improvement even with taking longer period.

Tongue atrophy – improve by decompression procedure.

Lingual atrophy is produced with the decrease of nerve function caused by the compression of 12th hypoglossal nerve. It will be recovered within one and half years by decompression method.

Visual field defect – developed by arterial sclerosis or arterial aneurysm.

There is a case where quarter hemianopia under nasal field with the compression of ocular nerve due to arterial sclerosis at internal carotid artery C2 region, and inferior hemianopia on ipsilateral eyesight is produced by meander compression at lateral anterior cerebral artery. Anterior communicating aneurysm is the one commonly experienced at clinic.

Medullary compression – clear sense of discomfort on contralateral half of the body.

Meandered and inflected vertebral artery strongly compress medulla oblongata. There is a case where patient appeals that rather than abnormal feeling at contralateral half body, something wrong in comparing with opposite side, so that under the patient wish and enough informed consent, surgical operation has been conducted. Complete cure has been gained by compression release of enough lift-up of vertebral artery with silk thread.

High-blood pressure – surgical operation is rare case

This is a case where surgery hasn’t been conducted for lowering blood pressure to difficult to control high-blood pressure, but it happens to be cured after the facial spasm. Recently, control of blood pressure becomes east to control with the usage of various kinds of antihypertensive drug so that it is considered that there are very few case where micro-vascular nerve decompression surgery may apply.

micro-vascular nerve decompression surgery (self-experiment example)
  Cases Complete cure rate (%)
Facial spasm 472 97
Trifacial neuralgia 157 92
glossopharyngeal neuralgia 5 80
Spastic torticollis 2 100
Dizziness 6 67
(100 improvement inclusive)
Oculomotor paralysis 14 100
Lingual atrophy 1 100
Visual field defect 1 100
medullary compression 1 100
*High-blood pressure 2 100

*The case happen to be cured after facial spasm surgery.

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