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Neurologic cause of weakness and paralysis, Distribution of weakness,…
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Distribution of weakness
Hemiparesis
- Hemiparesis results from an upper motor neuron lesion above the mid cervical spinal cord; most such lesions are above the foramen magnum. The presence of other neurologic deficits helps localize the lesion. Thus, language disorders, for example, point to a cortical lesion. Homonymous visual field defects reflect either a cortical or a subcortical hemispheric lesion. A “pure motor” hemiparesis of the face, arm, and leg often is due to a small, discrete lesion in the posterior limb of the internal capsule, cerebral peduncle in the midbrain, or upper pons. Some brainstem lesions produce “crossed paralyses,” consisting of ipsilateral cranial nerve signs and contralateral hemiparesis (Chap. 419). The absence of cranial nerve signs or facial weakness suggests that a hemiparesis is due to a lesion in the high cervical spinal cord, especially if associated with the Brown-Séquard syndrome
- hemiparesis，來自於UMN lesion above the mid cervical spinal cord, 大部分在枕骨大孔之上
- 其他的神經學症狀可幫助定位病灶點-->偏癱+神經學症狀=病灶位置(皮質or 內囊 or thalamas or 腦幹or脊髓)
- 語言能力喪失-->皮質
- Homonymous visual field defects-->cortical or a subcortical hemispheric lesion
- “pure motor” hemiparesis of the face, arm, and leg--> small, discrete lesion(lacunar infarction)in the posterior limb of the internal capsule, cerebral peduncle in the midbrain, or upper pons
- “crossed paralyses,”--同側cranial nerve signs＋對側偏癱--> 腦幹
- The absence of cranial nerve signs or facial weakness-->high cervical spinal cord, especially if associated with the Brown-Séquard syndrome
- Acute or episodic hemiparesis usually results from focal structural lesions, particularly rapidly expanding lesions, or an inflammatory process. Subacute hemiparesis that evolves over days or weeks may relate to subdural hematoma, infectious or inflammatory disorders (e.g., cerebral abscess, fungal granuloma or meningitis, parasitic infection, multiple sclerosis, sarcoidosis), or primary or metastatic neoplasms. AIDS may present with subacute hemiparesis due to toxoplasmosis or primary central nervous system (CNS) lymphoma. Chronic hemiparesis that evolves over months usually is due to a neoplasm or vascular malformation, a chronic subdural hematoma, or a degenerative disease
- acute or episodic
-focal structural lesions(ex. 中風)
-an inflammatory process
- Subacute hemiparesis that evolves over days or weeks
-SDH
-infectious or inflammatory disorders(e.g., cerebral abscess, fungal granuloma or meningitis, parasitic infection, multiple sclerosis, sarcoidosis)
-primary or metastatic neoplasms.
-AIDS may present with subacute hemiparesis due to toxoplasmosis or primary central nervous system (CNS) lymphoma
- Chronic hemiparesis that evolves over months
-neoplasm
-vascular malformation
-chronic subdural hematoma
-a degenerative disease
- Investigation of hemiparesis (Fig. 21-3) of acute origin starts with a computed tomography (CT) scan of the brain and laboratory studies. If the CT is normal, or in subacute or chronic cases of hemiparesis, magnetic resonance imaging (MRI) of the brain and/or cervical spine(including the foramen magnum) is performed, depending on the clinical accompaniments.
- Acute hemiparesis--> start with CT and 實驗室檢查
- 如果 CT正常or 這是一個 subacute or chronic cases of hemiparesis--> MRI + 臨床相關檢查
Paraparesis
- Acute paraparesis is caused most commonly by an intraspinal lesion, but its spinal origin may not be recognized initially if the legs are flaccid and areflexic. Usually, however, there is sensory loss in the legs with an upper level on the trunk, a dissociated sensory loss suggestive of a central cord syndrome (Chap. 434), or hyperreflexia in the legs with normal reflexes in the arms. Imaging the spinal cord (Fig. 21-3) may reveal compressive lesions, infarction (proprioception usually is spared), arteriovenous fistulas or other vascular anomalies, or transverse myelitis (Chap. 434).
- Acute paraparesis--> 最常見是脊髓內病灶
- 合併下肢sensory loss且上肢的嚴重程度較大, 感覺分離-->central cord syndrome
- or 下肢hyperreflexia with normal reflexes in the arms-->central cord syndrome
- Imaging the spinal cord (Fig. 21-3) may reveal compressive lesions, infarction (proprioception usually is spared), arteriovenous fistulas or other vascular anomalies, or transverse myelitis (Chap. 434).
- 待補
- Diseases of the cerebral hemispheres that produce acute paraparesis include anterior cerebral artery ischemia (shoulder shrug also is affected), superior sagittal sinus or cortical venous thrombosis, and acute hydrocephalus. Paraparesis may result from a cauda equina syndrome, for example, after trauma to the low back, a midline disk herniation, or an intraspinal tumor. The sphincters are commonly affected,
whereas hip flexion often is spared, as is sensation over the anterolateral thighs. Rarely, paraparesis is caused by a rapidly evolving anterior horn cell disease(such as poliovirus or West Nile virus infection), peripheral neuropathy (such as Guillain-Barré syndrome; Chap. 439), or myopathy (Chap. 441).
- Diseases of the cerebral hemispheres that produce acute paraparesis
-anterior cerebral artery ischemia (shoulder shrug also is affected)
-superior sagittal sinus or cortical venous thrombosis
-acute hydrocephalus
- Subacute or chronic spastic paraparesis is caused by upper motor neuron disease. When associated with lower-limb sensory loss and sphincter involvement, a chronic spinal cord disorder should be considered (Chap. 434). If hemispheric signs are present, a parasagittal meningioma or chronic hydrocephalus is likely. The absence of spasticity in a long-standing paraparesis suggests a lower motor neuron or myopathic etiology.
- Investigations typically begin with spi-nal MRI, but when upper motor neuron
signs are associated with drowsiness, confusion, seizures, or other hemispheric signs, brain MRI should also be performed, sometimes as the initial
investigation. Electrophysiologic studies are diagnostically helpful when clinical findings suggest an underlying neuromuscular disorder.
Quadriparesis or Generalized Weakness
- Generalized weakness may be due to disorders of the CNS or the motor unit. Although the terms often are used interchangeably, quadriparesis is commonly used when an upper motor neuron cause is suspected, and generalized weakness is used when a disease of the motor units is likely. Weakness from CNS disorders usually is associated with changes in conscious-ness or cognition and accompanied by spasticity, hyperreflexia, and sensory disturbances. Most neuromuscular causes of generalized weakness are associated with normal mental function, hypotonia, and hypoactive muscle stretch reflexes. The major causes of intermittent weakness are listed in Table 21-2. A patient with generalized fatigability without objective weakness may have the chronic fatigue syndrome
ACUTE QUADRIPARESIS
- Quadriparesis with onset over minutes may
result from disorders of upper motor neurons (such as from anoxia, hypotension, brainstem or cervical cord ischemia, trauma, and systemic metabolic abnormalities) or muscle (electrolyte disturbances,certain inborn errors of muscle energy metabolism, toxins, and periodic
paralyses). Onset over hours to weeks may, in addition to these disor-ders, be due to lower motor neuron disorders such as Guillain-Barré syndrome (Chap. 439).
- In obtunded patients, evaluation begins with a CT scan of the brain. If upper motor neuron signs are present but the patient is alert, theinitial test is usually an MRI of the cervical cord. If weakness is lower motor neuron, myopathic, or uncertain in origin, the clinical approach begins with blood studies to determine the level of muscle enzymes and electrolytes and with EMG and nerve conduction studies.
SUBACUTE OR CHRONIC QUADRIPARESIS
- Quadriparesis due to upper motor neuron disease may develop over weeks to years from chronic myelopathies, multiple sclerosis, brain or spinal tumors, chronic sub-
dural hematomas, and various metabolic, toxic, and infectious disor-ders. It may also result from lower motor neuron disease, a chronic neuropathy (in which weakness is often most profound distally), or myopathic weakness (typically proximal).
- When quadriparesis develops acutely in obtunded patients, evaluation begins with a CT scan of the brain. If upper motor neuron signs have developed acutely but the patient is alert, the initial test is usually an MRI of the cervical cord. When onset has been gradual, disorders of the cerebral hemispheres, brainstem, and cervical spinal cord can usually be distinguished clinically, and imaging is directed first at the clinically suspected site of pathology. If weakness is lower motor neuron, myopathic, or uncertain in origin, laboratory studies to determine the levels of muscle enzymes and electrolytes, and EMG and nerve conduction studies help to localize the pathologic process.
Monoparesis
- Monoparesis usually is due to lower motor neuron disease, with or without associated sensory involvement. Upper motor neuron weakness occasionally presents as a monoparesis of distal and nonantigravity muscles. Myopathic weakness rarely is limited to one limb.
ACUTE MONOPARESIS
- If weakness is predominantly distal and of upper motor neuron type and is not associated with sensory impairment or pain, focal cortical ischemia is likely (Chap. 420); diagnostic possibilities are similar to those for acute hemiparesis. Sensory loss and pain usually accompany acute lower motor neuron weakness; the weakness commonly localizes to a single nerve root or peripheral nerve, but occasionally reflects plexus involvement. If lower motor neuron weakness is likely, evaluation begins with EMG and nerve conduction studies.
SUBACUTE OR CHRONIC MONOPARESIS
- Weakness and atrophy that develop over weeks or months are usually of lower motor neuron origin. When associated with sensory symptoms, a peripheral cause (nerve, root, or plexus) is likely; otherwise, anterior horn cell disease should be considered. In either case, an electrodiagnostic study is indicated. If weakness is of the upper motor neuron type, a discrete cortical (precentral gyrus) or cord lesion may be responsible, and appropriate imaging is performed.
Distal Weakness
- Involvement of two or more limbs distally
suggests lower motor neuron or peripheral nerve disease. Acute distal lower-limb weakness results occasionally from an acute toxic polyneuropathy or cauda equina syndrome. Distal symmetric weakness usually develops over weeks, months, or years and, when associated with numbness, is due to peripheral neuropathy (Chap. 438). Anterior horncell disease may begin distally but is typically asymmetric and without accompanying numbness (Chap. 429). Rarely, myopathies present with distal weakness (Chap. 441). Electrodiagnostic studies help localize the disorder (Fig. 21-3).
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Proximal Weakness
- Myopathy often produces symmetric weakness of the pelvic or shoulder girdle muscles (Chap. 441). Diseases of the neuromuscular junction, such as myasthenia gravis (Chap. 440), may present with symmetric proximal weakness often associated with pto-sis, diplopia, or bulbar weakness and fluctuating in severity during the day. In anterior horn cell disease, proximal weakness is usually asymmetric, but it may be symmetric if familial. Numbness does not occur with any of these diseases. The evaluation usually begins with determination of the serum creatine kinase level and electrophysiologic studies.
Weakness in a Restricted Distribution
- Weakness may not fit any of these patterns, being limited, for example, to the extraocular, hemifacial, bulbar, or respiratory muscles. If it is unilateral, restricted weakness usually is due to lower motor neuron or peripheral nerve dis-ease, such as in a facial palsy. Weakness of part of a limb is commonly due to a peripheral nerve lesion such as an entrapment neuropathy. Relatively symmetric weakness of extraocular or bulbar muscles frequently is due to a myopathy (Chap. 441) or neuromuscular junction disorder (Chap. 440). Bilateral facial palsy with areflexia suggests Guillain-Barré syndrome (Chap. 439). Worsening of relatively symmet-ric weakness with fatigue is characteristic of neuromuscular junction disorders. Asymmetric bulbar weakness usually is due to motor neu-ron disease. Weakness limited to respiratory muscles is uncommon and usually is due to motor neuron disease, myasthenia gravis, or polymy-ositis/dermatomyositis (Chap. 358).
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Pathogenisis
Upper Motor Neuron Weakness
- Lesions of the upper motor neurons or their descending axons to the spinal cord (Fig. 21-1) produce weakness through decreased activation of lower motor neurons.
- UMN lesion造成對下游的神經活化減少-->weakness
- In general, distal muscle groups are affected more severely than proximal ones, and axial movements are spared unless the lesion is severe and bilateral. Spasticity is typical but may not be present acutely. Rapid repetitive movements are slowed and coarse, but normal rhythmicity is maintained. With corticobulbar involvement, weakness occurs in the lower face and tongue; extraocular, upper facial, pharyngeal, and jaw muscles are typically spared. Bilateral corticobulbar lesions produce a pseudobulbar palsy: dysarthria, dysphagia, dysphonia, and emotional lability accompany bilateral facial weakness and a brisk jaw jerk. Electromyogram (EMG) (Chap. 438) shows that with weakness of the upper motor neuron type, motor units have a diminished maximal discharge frequency.
- 遠端肌肉被影響的較嚴重，axial movement(軸向運動)通常不受影響除非雙側且嚴重
- Spasticity，在急性期可能不表現
Lower Motor Neuron Weakness
- This pattern results from disorders of lower motor neurons in the brainstem motor nuclei and the anterior horn of the spinal cord or from dysfunction of the axons of these neurons as they pass to skeletal muscle (Fig. 21-2). Weakness is due to a decrease in the number of muscle fibers that can be activated through a loss of α motor neurons or disruption of their connections to muscle. Loss of γ motor neurons does not cause weakness but decreases tension on the muscle spindles, which decreases muscle tone and attenuates the stretch reflexes. An absent stretch reflex suggests involvement of spindle afferent fibers.
- 位置: 1. 腦幹的運動神經核 2. 脊髓的前角 3.以上兩類到肌肉的傳導路徑（axon）
- α motor neurons受損 or 傳遞到肌肉的路徑（axon）受損-->被活化的肌肉細胞數目減少-->無力
- Loss of γ motor neurons -->decreases tension on the muscle spindles-->decreases muscle tone and attenuates the stretch reflexes ps. γ motor neurons受損跟weakness無關
- When a motor unit becomes diseased, especially in anterior horn
cell diseases, it may discharge spontaneously, producing fasciculations. When α motor neurons or their axons degenerate, the denervated muscle fibers also may discharge spontaneously. These single muscle fiber discharges, or fibrillation potentials, cannot be seen but can be recorded with EMG. Weakness leads to delayed or reduced recruitment of motor units, with fewer than normal activated at a particular discharge frequency.
- fasciculation： 受損的motor units（LMN or muscle fiber) 可能自發性放電
Neuromuscular Junction Weakness
- Disorders of the neuromuscular junctions produce weakness of variable degree and distribution. The number of muscle fibers that are activated varies over time, depending on the state of rest of the neuromuscular junctions. Strength is influenced by preceding activity of the affected muscle. In myasthenia gravis, for example, sustained or repeated contractions of affected muscle decline in strength despite continuing effort (Chap. 440). Thus, fatigable
weakness is suggestive of disorders of the neuromuscular junction, which cause functional loss of muscle fibers due to failure of their activation.
- NMJ disease. ex自體免疫（重症肌無力症）, 毒素（肉毒桿菌, aminoglycoside）
- 無力影響的範圍和程度各不相同
- 肌肉張力, 大小, 及反射都正常
- 主要症狀：重複運動後會無力, 最常見影響眼球肌肉及延髓肌肉
Myopathic Weakness
- Myopathic weakness is produced by a decrease in the number or contractile force of muscle fibers activated within motor units. With muscular dystrophies, inflammatory myopathies, or myopathies with muscle fiber necrosis, the number of muscle fibers is reduced within many motor units. On EMG, the size of each motor unit action potential is decreased, and motor units must be recruited more rapidly than normal to produce the desired power. Some myopathies produce weakness through loss of contractile force of muscle fibers or through relatively selective involvement of type II (fast) fibers. These myopathies may not affect the size of individual motor unit action potentials and are detected by a discrepancy between the electrical activity and force of a muscle.
- motor unit裡muscle fiber的數目 or 收縮的力度減少-->無力
Psychogenic Weakness
Weakness may occur without a recognizable organic basis. It tends to be variable, inconsistent, and with a pattern of distribution that cannot be explained on a neuroanatomic basis. On formal testing, antagonists may contract when the patient is supposedly activating the agonist muscle. The severity of weakness is out of keeping with the patient’s daily activities.
- 神經解剖學無法解釋的weakness分布
- weakness的嚴重程度跟pt 的日常活動不符
