MindMap Gallery Neurology Chapter 6 Peripheral Nerve Diseases
Neurology Chapter 6 Peripheral Nerve Disease Mind Map Peripheral nerve disease refers to diseases originating from structural or functional damage to the peripheral nervous system.
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Neurology Chapter 6 Peripheral Nerve Diseases
Section 1 Overview
【anatomy】
spinal nerve
Anatomy and Physiology
Mixed: somatosensory fibers, somatic motor fibers, visceral sensory fibers, visceral motor fibers
Each spinal nerve trunk is divided into anterior branch, posterior branch, meningeal branch and communicating branch immediately after exiting the intervertebral foramen.
Segmental distribution of skin: T2 → sternal angle; T4 → nipple level; T6 → xiphoid process level; T8 → lower edge of costal arch; T10 → umbilical level; T12 → groin level; in the distribution of nerves in the limbs, the upper and lower ones It is distributed in the proximal part near the trunk, and the middle nerve is distributed in the distal part of the limbs.
Lesion location
Movement disorders: Anterior root injury alone causes movement disorders, while nerve plexus/nerve trunk injuries can also involve sensory and autonomic nerves.
Irritation symptoms: fasciculations, muscle spasms, muscle spasms
Paralytic symptoms: weakening/loss of muscle strength, muscle atrophy
Sensory impairment: Posterior root damage is segmentally distributed sensory impairment accompanied by severe radicular pain; nerve plexus and nerve trunk damage is distribution area sensory impairment accompanied by pain and motor/autonomous nerve dysfunction; nerve ending damage is symmetrical at the distal end of the limb Sexually distributed glove-sock-like sensory impairment with motor and autonomic dysfunction
Reflection changes: shallow reflection and deep reflection weaken/disappear
Autonomic nervous system dysfunction: hyperhidrosis/anhidrosis, pale mucous membranes/cyanosis, decreased skin temperature, skin edema, subcutaneous tissue atrophy, hyperkeratosis, pigmentation, skin ulcers, hair loss, nail gloss loss, nail brittleness, sexual functional impairment, vesicorectal dysfunction, orthostatic hypotension, decreased lacrimal gland secretion
Others: action tremor, peripheral nerve enlargement, deformity, nutritional disorders
autonomic nerve
Anatomy and Physiology
Central autonomic nervous system: Parasympathetic nuclei of the cerebral cortex/hypothalamus/brainstem, lateral horn areas of each spinal cord segment
Peripheral autonomic nerves
Sympathetic nervous system: C8~L2 spinal cord lateral horn neurons → sympathetic trunk paravertebral ganglia, celiac ganglia → postganglionic fibers distribute along with spinal nerves to sweat glands, blood vessels, smooth muscles, and internal organs
Parasympathetic nervous system: brainstem S2~S4 spinal cord lateral horn neurons → ganglia near organs/intra-organs → postganglionic fibers innervate pupillary sphincter, ciliary muscle, submandibular gland, sublingual gland, lacrimal gland, parotid gland, tracheobronchi, viscera
Lesion location
Sympathetic nerve damage-parasympathetic nerve hyperfunction: miosis, increased salivation, slowed heart rate, dilated blood vessels, decreased blood pressure, increased gastrointestinal motility and digestive gland secretion
Parasympathetic nerve damage - Sympathetic nerve hyperfunction: miosis, exophthalmos, accelerated heart rate, vasoconstriction
contraction, increased blood pressure, accelerated respiration, bronchiectasis, gastrointestinal motility and inhibition of digestive gland secretion
[Cause and pathology]
1. Wallerian degeneration/anterograde degeneration: refers to the cessation of axoplasmic flow after any trauma breaks the axon, and the axon and myelin sheath distal to the severed end quickly degenerate and dissolve (developing from proximal to distal). It is phagocytized by Schwann cells/macrophages; degeneration proximal to the stump is only 1 to 2 Ranvier nodes and no longer progresses; the cell body swells and moves to the edge, and Nissl bodies are dissolved. When the lesion is close to the cell body, nerve cell death
2. Axonal degeneration: It is a common pathological change in toxic, metabolic, and immune-mediated inflammatory neuropathies; it is mainly caused by the failure of cell body protein synthesis/axonal transport disorders and the inability to supply distal axonal nutrition. Degeneration, myelin fragmentation; lesions usually develop from distal to proximal axon
3. Segmental demyelination (segmental demyelination): refers to the localized myelin sheath destruction on nerve fibers. The axons are basically normal, but the nerve conduction speed slows down; the lesions are often spotty, and some myelin sheaths are destroyed. Some are normal; common in peripheral neuropathy caused by GBS, lead poisoning, diphtheria, etc.
4. Neuronal cell degeneration
[Clinical manifestations]
Basic performance
Pain - neuralgia (neuralgia): pain occurs in the distribution area of the affected sensory nerve, which is like electric shock/burning; the nerve conduction function is normal and there is no obvious change in the main substance of the nerve.
Others: numbness, hypoesthesia, muscle weakness, and muscle atrophy; patients with peripheral neuropathy, mainly axonal degeneration, often have no pain or abnormal sensation.
Classification
Course: acute, subacute, chronic, recurrent, progressive Symptoms: sensory, motor, mixed, autonomic Site: radiculopathy, plexopathy, neuropathic disease
Commonly used in clinical practice: symmetric polyneuropathy, mononeuropathy, major mononeuropathy
Section 2 Neuralgia
1. (Primary) trigeminal neuralgia
[Clinical manifestations]
Features
It is more common in middle-aged and elderly women over 40 years old; often limited to 1/2 branches of the unilateral trigeminal nerve (especially the maxillary nerve and mandibular nerve); as the disease progresses, the number of attacks gradually increases, the attack time gradually extends, the intermittent period shortens, and even It is a persistent attack that rarely heals on its own.
Performance
Pain: During the attack, it manifests as severe electric shock-like, acupuncture-like, knife-like, tearing, and burning pain in the upper, lower jaw, and tongue areas of the cheeks, mostly unilateral, lasting 1 to 2 seconds, sudden and intermittent. The symptoms are completely normal; the number of attacks is less at the beginning, and then increases and the pain becomes stronger.
Trigger points/trigger points: The corners of the patient's mouth, nose, cheeks, and tongue are sensitive areas that can induce pain with a light touch.
Painful convulsions (tic douloureux): In severe cases, facial muscle reflex twitching may occur due to pain, and the corners of the mouth may be pulled to the affected side; accompanied by facial skin redness, tears, and salivation.
Nervous system examination: generally no positive signs (this is an important feature of primary trigeminal neuralgia)
[Differential diagnosis] Secondary trigeminal neuralgia: pain is persistent, accompanied by facial hypoesthesia and dull corneal reflex on the affected side, often combined with other symptoms of cranial nerve damage (with corresponding positive signs); seen in MS, bulbar syrinx, craniocerebral bottom tumor
【treat】
medical treatement
Carbamazepine: drug of choice; contraindicated in pregnant women
Others: Phenytoin, Clonazepam, Amitriptyline, Baclofen
Other treatments
Percutaneous semilunar ganglion radiofrequency thermocoagulation therapy: suitable for long-term and medical treatment ineffective/intolerable, elderly and frail patients with systemic diseases who cannot tolerate surgery
Surgical treatment: microvascular decompression, partial sensory rhizotomy of trigeminal nerve, gamma knife treatment
2. Sciatica
[Clinical manifestations]
root nature
Multiple acute/subacute onset, initially lower back pain/waist stiffness and discomfort
Pain: Typical manifestations are burning/knife-like pain that radiates from the waist to one hip, back of the thigh, popliteal fossa, outside of the calf, and instep. On top of the persistent pain, there are episodic intensifications, especially at night. , aggravated by coughing, sneezing, or straining to defecate; there is often tenderness (but not obvious) in the lumbar spinous process/transverse process at the level of the lesion; special pain relief postures may be used
You may feel pins and needles and numbness on the outer side of the calf and dorsum of the foot on the affected side; the muscle strength of the toe extension/flexion function is weakened, and the ankle reflex is weakened/disappeared
Diagnostic tests: Laségue sign, positive geniothoracic test
Dry
Multiple subacute/chronic onset
Pain: Mainly distributed in the sciatic nerve pathway, the waist discomfort is not obvious, but there are tender points (paralumbar points, hip points, popliteal points, gastrocnemius points, ankle points); special pain relief postures may be used
The sensory impairment on the lateral side of the calf and the dorsum of the foot on the affected side is more obvious; the muscles in the area controlled by the sciatic nerve are relaxed with mild muscle atrophy, and the ankle reflex is weakened/disappeared
Diagnostic test: Laségue syndrome is often positive
Section 3 Mononeuropathy
1. Idiopathic facial paralysis/facial neuritis/Bell's palsy
[Definition] Acute peripheral facial paralysis caused by non-specific inflammation of the facial nerve in the stylomastoid foramen
[Cause] ① Entrapment neuropathy: inflammation at the stylomastoid foramen outlet of the facial nerve causing compression of the facial nerve; ② Genital ganglionitis after herpes zoster virus infection; ③ Lyme disease; ④ Granuloma
[Clinical manifestations]
Features
More common in 20 to 40 years old, more common in men; onset is acute, and facial nerve paralysis reaches its peak within hours to days
Performance
Peripheral facial paralysis on the affected side: disappearance of forehead lines, inability to frown, inability to close eye fissures, positive Bell sign/Bell phenomenon (weakness in closing eyes on the affected side → the eyeball moves upward and outward when closing the eyes forcefully to expose the white sclera under the cornea), nose Lip grooves become shallower, mouth corners droop/deviate to the opposite side, air leakage when blowing/whistling
Localization manifestations: Lesions above the chorda tympani may cause loss of taste in the front 2/3 of the ipsilateral tongue; lesions above the stapedius nerve may cause loss of taste in the front 2/3 of the ipsilateral tongue and hyperacusis.
Some people may have persistent pain behind the ear and mastoid tenderness on the affected side 1 to 2 days before the onset of illness.
Hunt syndrome: When the geniculate ganglion is involved, in addition to peripheral facial paralysis on the affected side, loss of taste in the front 2/3 of the tongue, and hyperacusis, there may also be mastoid pain, auricle/external auditory canal sensory disturbance, and external auditory canal/tympanic membrane herpes.
Sequelae: Mainly caused by regenerated nerve fibers growing into adjacent nerve sheath cell pathways
Abnormal gustatory tear reflex: crocodile tears, accompanied by temporal skin flushing, fever, and sweat secretion
Combined movements: For example, when blinking, the upper lip on the affected side trembles slightly; when teeth are exposed, the eye on the affected side closes involuntarily; when the eyes are closed, the frontalis muscle on the affected side contracts and the nasolabial fold deepens
Paralytic muscle contracture and hemifacial spasm on the affected side: At this time, the eye fissures on the affected side shrink, the nasolabial groove becomes deeper, and the corners of the mouth are tilted toward the affected side.
【Differential Diagnosis】
1.GBS: multiple bilateral peripheral facial paralysis, accompanied by symmetrical limb flaccid paralysis and sensory impairment; cerebrospinal fluid examination shows characteristic protein-cell separation phenomenon
2. Otogenic facial nerve palsy: clear primary disease and special symptoms
3. Posterior fossa tumor/meningitis: slow onset, accompanied by other symptoms of cranial nerve damage and special manifestations of the primary disease
4. Diabetic neuropathy: single or multiple cranial nerves may be damaged (oculomotor nerves and abducens nerves may also be damaged)
5. Neurological Lyme disease: unilateral/bilateral facial nerve paralysis, often accompanied by fever and migrating erythema of the skin; often involving other cranial nerves
[Treatment] Drug treatment (glucocorticoids, vitamin B, acyclovir; facial nerve conduction velocity test after 1 week can help determine prognosis), physical therapy, eye protection, rehabilitation treatment, surgery
2. Hemifacial spasm/facial twitching
[Characteristics] Primary cases are mostly caused by the facial nerve being compressed by vascular loops; it manifests as intermittent involuntary clonic twitching/painless rigidity of one side of the facial muscles without other positive signs of the nervous system; in the early stage, it is mostly caused by the orbicularis oculi muscle. Later, it slowly spreads to other facial muscles on one side of the face, with perioral muscle twitching being the most obvious; a few may be accompanied by facial myoparesis in the late stages of the disease; local injection of botulinum toxin A is the first choice for palliative treatment.
Section 4 Polyneuropathy
1. Polyneuropathy/Peripheral Neuropathy
[Characteristics] Multiple nerve damage in the distal limbs; the most common pathological change is axonal degeneration, presenting as "dying-back neuropathy"①; clinical manifestations include symmetrical movement disorders in the distal limbs (lower motor nerves) paralysis), sensory impairment (glove-sock-like distribution), autonomic nervous system dysfunction, extending from far to near; EMG shows neurogenic damage, and nerve conduction velocity is reduced to varying degrees.
① When the distal peripheral nerve is injured, due to insufficient supply of axoplasmic flow in the distal nerve, secondary nerve cell protein synthesis is inhibited, causing peripheral nerve degeneration to progress from far to near.
2. Guillain-Barré syndrome (GBS)
[Definition] It is an autoimmune-mediated peripheral neuropathy that mainly damages most spinal nerve roots and peripheral nerves, and often involves cranial nerves. The clinical characteristics are acute onset, with symptoms usually peaking around 2 weeks, manifested by multiple nerve roots and Peripheral nerve damage, often with cerebrospinal fluid protein-cell separation, usually has a single-phase self-limiting course
[Cause] May be related to Campylobacter jejuni (CJ), CMV, Epstein-Barr virus, Mycoplasma pneumoniae, hepatitis B virus, and HIV infection
[Clinical manifestations]
【Auxiliary inspection】
hematology
Generally normal; anti-ganglioside antibodies ( ), CJ antibodies ( ) may be present
cerebrospinal fluid
The number of white blood cells is normal; there is protein-cell separation phenomenon (protein ↑ but <1.0g/L, cells are normal; most obvious in the 3rd to 6th week after onset); the IgG index is elevated, but there are oligoclonal IgG bands in both serum and cerebrospinal fluid; Some may have anti-ganglioside antibodies ( )
electrophysiological examination
Suggests demyelinating disease of peripheral nerves
Sural nerve biopsy
Auxiliary diagnostic method, but not required examination
【diagnosis】
Internationally recommended diagnostic criteria: ① Progressive limb muscle weakness with loss of tendon reflexes; ② The course of the disease gradually worsens for several days to 4 weeks; ③ The signs of the limbs on both sides are basically symmetrical; ④ The sensory symptoms and signs are mild; ⑤ There is no fever during acute onset, The course of the disease progresses and begins to gradually recover after 2 to 4 weeks; ⑥ cerebrospinal fluid cells are <10×106/L and protein content increases; ⑦ electrophysiological examination shows slowing down of nerve conduction velocity/conduction block
The diagnosis of GBS is generally not supported if the following symptoms occur: ① Significant and persistent asymmetric limb weakness; ② Bladder/rectal dysfunction as the first manifestation or persistent bladder/rectal dysfunction; ③ The number of cerebrospinal fluid mononuclear cells is >50 × 106/L; ④ Segmented nuclear leukocytes appear in cerebrospinal fluid; ⑤ Limb paralysis with clear sensory level
【Differential Diagnosis】
1. Acute poliomyelitis: fever is common, limb paralysis is usually limited to one lower limb, and there is no sensory impairment or cranial nerve damage; cerebrospinal fluid protein and cell number are increased.
2. Acute transverse myelitis: There is a history of fever during the onset period, paraplegia occurs in 1 to 2 days, and movement disorders below the damaged level are accompanied by tract sensory disturbances; bowel and bowel disorders occur in the early stage, and the cranial nerves are not affected.
3. Hypokalemic periodic paralysis: rapid onset of flaccid paralysis of the limbs, no sensory impairment, respiratory muscles and cranial nerves are generally not affected; cerebrospinal fluid is normal, serum potassium↓; can recur; potassium supplementation is effective
4. Generalized myasthenia gravis: morbid fatigue of affected skeletal muscles, fluctuating symptoms, light and heavy in the morning and evening; neostigmine test ( )
5. Others: muscle paralysis and peripheral neuropathy caused by botulism, diphtheria, porphyria, AIDS, diabetes, etc.
【treat】
3. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)/chronic GBS
[Characteristics] It is a group of immune-mediated inflammatory demyelinating diseases with a chronic progressive or relapsing course; the onset is insidious, and precursor infection is rare before the onset; the main manifestation is symmetrical limb weakness, which develops from the distal end to the proximal end. , accompanied by weakened tendon reflexes, but generally does not cause dyspnea; peripheral sensory loss in the limbs, and some may have autonomic nervous dysfunction; the progression period of the disease is >8w, and the disease course is divided into chronic monophasic type, chronic relapsing type, step-progressive type, chronic Progressive type; most respond well to immunotherapy (such as hormones)
4. Polyneuropathy
[Characteristics] Clinical symptoms caused by simultaneous/sequential damage to multiple cranial nerves on one or both sides; such as superior orbital fissure syndrome (involving cranial nerves III, IV, V1, and VI), cavernous sinus syndrome (except for the superior orbital fissure) In addition to the cranial nerves of the syndrome, phenomena such as proptosis of the eye on the affected side, congestion and edema of the eyelid conjunctiva, and congestion and edema of the eyelid conjunctiva, and venous obstruction in the cavernous sinus may occur), orbital apex syndrome (involving cranial nerves II, III, IV, V1, and VI). ), petrosal apex syndrome (involving cranial nerves VI and V1), cerebellopontine angle syndrome (involving cranial nerves V, VII, and VIII), jugular foramen syndrome (involving cranial nerves IX, X, and XI)