Speech disadvantage + dysarthria, don't just think of cerebral infarction!

*For medical professionals only

For this case, the director raised 7 questions .

When there is a speech disadvantage with dysarthria, what else can be thought of other than cerebral infarction? Take a look at this classic case

Let's look at the case information first

The patient, a 68-year-old female, was admitted to the hospital

The symptoms gradually worsened, and he was admitted to the hospital before October, and the head MRI showed bilateral frontal parietal lobe, radial crown, small ischemic focus in the basal ganglion, sparse white matter and cerebral atherosclerosis changes, and cerebral infarction was diagnosed, and the symptoms were alleviated after symptomatic treatment

After the patient's above symptoms are still gradually aggravated, the speech cannot be heard by others, the solid food cannot be swallowed, the drinking water choking cough is more frequent than before, and no special treatment

Confusion, dysarthria, atrophy and fibrillation of the tongue muscles, decreased gag reflex, positive mandibular reflex, perioral reflex, and left palmomandibular reflex;

Right sternocleidomastoid muscle, bilateral trapezius reflex, bilateral deltoid reflex positive, bilateral biceps tendon reflex, triceps tendon reflex and bilateral radioperiosteal reflex, positive bilateral fascial spinal and bilateral rectus reflexes, positive bilateral gluteus maximus reflexes, bilateral knee tendon reflexes and Achilles tendon reflexes, ankle-clonic (+), positive bilateral pathological signs

The volume of the bibiapial interstitial muscles, interosseous muscles, bilateral deltomi, inferior ganglion muscles, left erect spinal muscles, and bi-lower limb quadriceps muscles decreased
.

Limb muscle tone is normal, bilateral Babinski sign positive
.

Ancillary tests:

Blood, urine and stool routine, all biochemical items, tumor full items, six items of A Gong, and five items of coagulation did not see obvious abnormalities; MRI of the skull and cervical vertebrae: chronic ischemic brain changes, small softening foci
in the right basal ganglia.

C4-5, C5-6 discs are mildly herniated
.

Cervical vertebrae hyperplasia and degeneration
.

Director rounds, the problem comes.
.
.

Q1: When you see dysarthria, what comes to mind?
Dysarthria refers to weakened or paralyzed muscle strength of the pronounced muscles, altered muscle tone, or poor coordination, resulting in inaccurate pronunciation, uneven proverbial prosody, slow speech, and rhythmic disturbances
.
It is very different
from aphasia with normal speech and incorrect grammar.

The diagnosis of dysarthria is mainly to identify the type of dysarthria and determine its cause
.

1) Retardant dysarthria: due to the myopathy of the vocal muscle itself, or the damage of the lower motor neurons innervating the vocal muscle, the flaccid muscle is weak
.
A.
Acute flaccid dysarthria: can be caused
by cerebrovascular disease, inflammation, multiple sclerosis, toxic diseases, etc.
B.
Subacute flaccid dysarthria: more common in myasthenia gravis caused by medulla oblonga palsy, the onset is mostly in adolescents, dysarthria is fluctuating
.

Fatigue is aggravated when it is aggravated, and it improves after rest, and there is a heavy phenomenon
in the morning and in the afternoon.

Symptoms of injection of neostigmine and ephenol chloride may temporarily improve
significantly.

Accompanied by dysphagia, chewing, and weakness
of the eye, extremities, and trunk muscles.
C.
Chronic progressive flaccid dysarthria: common in the following amyotrophic lateral sclerosis, simultaneous degeneration of upper and lower motor neurons, such as lesions dominated by the cortical medulla tract, dysarthria is often spasmodic; If the lesion is predominantly subnuclear cranial nerve in the medulla oblongata, the dysarthria is flaccid
.

Onset is slow and gradually worsened, with atrophy and weakness of the face, masticatory muscles, tongue, pharynx, jaw, and trunk limbs, and due to simultaneous damage to the pyramidal bundle, the tendon reflexes are often hyperactive, and the pathological reflexes are positive
.

Paralysis of the lower motor element, progressive atrophy of the muscles, fibrillation of the muscle bundles, and no sensory impairment
.
2) Spasmodic twin dysarthria: caused by damage to the bilateral cortical medulla oblongata (superior motor neurons) innervating the vocal muscles, which increases the muscle tone and weakens the mouth-tongue-lip muscle, also known as pseudoprolongation paralysis
.

Speech is delayed, the tone is low and the nasal tone is heavy
.
Amuscular atrophy, often accompanied by strong crying and laughter, hyperreflexia of the lower jaw, positive palmomandibular reflex, dysphagia, spasmodic paralysis of both limbs, etc.
, most commonly found in bilateral cortical nuclei bundles
.

Patients caused by motor neuron disease are often accompanied by symptoms and signs
of bilateral limb progressive upper and lower motor neuron damage.
3) Dysarthria: often caused
by cerebellar or extrapyramidal lesions.
Good article recommendation: aphasia and dysarthria, silly and unclear? Q2: What diseases can you think of when the atrophy and weakness of the muscles innervated by the upper and lower motor neurons are accompanied by dysarthria?
Motor neuron disease, Lambert-Eaton myasthenia syndrome, cervical or lumbar spondylosis, medulla oblongata and syringomyelia, cervical spinal cord tumors, multifocal motor neuropathy, Hirayama disease, spinal muscular atrophy, Kennedy disease, hereditary spastic paraplegia, paraneoplastic syndrome.
.
.

Q3: According to the chronic course of the patient, what is the first consideration of the cause?
In this case, the patient has symptoms such as slurred speech and dysphagia, which are characterized by progressive exacerbations
.
Examination shows that there is damage to upper and lower motor neurons in the brain stem and neck, chest, and waist: 1) signs of damage to upper motor neurons:

• Brainstem: positive mandibular reflex, bilateral perioral reflex, left palmomandibular reflex
  .
  
  
• Cervical segment: right sternocleidomastoid muscle, bilateral trapezius muscle reflex, bilateral deltoid reflex positive, bilateral biceps tendon reflex, triceps tendon reflex, bilateral radioperiosteal hyperreflex
  .
  
  
• Thoracic segment: positive
  bilateral spinal muscle reflexes and bilateral rectus abdominal reflexes.
  
  
• Lumbar segment: bilateral knee tendon reflex, bilateral hyperreflexia Achilles tendon, positive bilateral glutes, ankleclonic (+), more pronounced on the left than the right, positive bilateral pathological signs
  .
  
  

2) Signs of lower motor neuron damage:

• Brainstem: tongue muscle atrophy with fibrillation
  .
  
  
• Cervical segment: bilateral inferior posterior muscle atrophy, left side is more pronounced, bilateral deltoid muscle atrophy, large and small fish muscle and interosseous muscle atrophy
  .
  
  
• Thoracic segment: atrophy of the left erectus muscle
  .
  
  
• Lumbar segment: quadriceps atrophy
  .
  
  

There is currently a high probability of considering motor neuron disease according to physical
examination.
Q4: What are the classic manifestations of motor neuron disease ^[1]?
(1) Progressive muscular atrophy: the damage is limited to the anterior horn cells of the spinal cord, which is manifested by weakness and muscle atrophy without cone bundle signs
.
(2) Progressive medulla oblongature: the movement nucleus of the medulla oblongata is damaged separately and manifested as weakness and atrophy of the throat and tongue muscles
.
(3) Primary lateral sclerosis: only the cone bundle is involved and manifests as weakness and cone bundle signs
.
(4) Amyotrophic lateral sclerosis (ALS): Upper and lower motor neurons are damaged, and those who show muscle weakness, muscle atrophy and pyramidal banding signs are ALS
.
However, many cases first show one type of manifestation, and then another type evolves into ALS
.

Therefore, it is sometimes difficult to determine which type
is in the early stages of the disease.
At this time, the results of the electroneurogram return: 1.
Nerve conduction speed:
the left median nerve and the bilateral fibula total nerve motor velocity conduction amplitude decreases, and the left tibial nerve motor conduction proximal amplitude decreases; Yu was examined for the normal range of nerve motor conduction and normal range
for nerve sensory conduction to be examined.
2.
Reflexology: bilateral tibial nerve H reflex, F wave determination normal range
.
3.
Electromyography of the left tibial anterior muscle, the left medial femoral muscle, and the right little finger excisculars muscle are neurogenicity; Autopotential can be seen during the resting phase of the left T10 paraspinal muscle, suggesting the possibility of neurogenic damage; The time limit of the left tongue muscle MUP (motor action potential) is widened and the amplitude is increased, suggesting neurogenic damage that is not excluded; No abnormal changes
were seen in the muscles examined for Yu.
Conclusion: Extensive neurogenic damage?
Q5: What are the basic conditions for ALS diagnosis?
(1) The diagnosis of ALS must meet the following 3 points: a.
Clinical
, neurophysiological or pathological examination confirms evidence of
lower motor neuron involvement.
b.
Clinical examination shows evidence
of upper motor neuron involvement.
c.
Progressive progression of the condition: through medical history, physical examination, or electrophysiological examination, clinical symptoms or signs confirm that clinical symptoms or signs are developing sexually in one area, or from one area
to another.
(2) At the same time, the following 2 points
must be excluded a.
Electrophysiological or pathological examination suggests that the patient may have other diseases
that cause upper and lower motor neuron lesions.
b.
Neuroimaging suggests that the patient may have other disorders
that cause the above clinical or electrophysiological changes.

Q6: What are the diagnostic criteria for AIS?
(1) Clinically confirmed ALS: through clinical or neuroelectrophysiological examination, it is confirmed that there is evidence
of simultaneous involvement of upper and lower motor neurons in at least 3 of the 4 regions.
(2) Clinical proposed diagnosis of ALS: through clinical or neuroelectrophysiological examination, it is confirmed that there is evidence
of simultaneous involvement of upper and lower motor neurons in at least 2 of the 4 regions.
(3) Clinically possible ALS: through clinical or neuroelectrophysiological examination, it is confirmed that only 1 region has evidence of simultaneous involvement of upper and lower motor neurons, or only evidence of upper motor neuron involvement in 2 or more regions
.

Imaging and laboratory tests have been performed to rule out other disorders
.
Q7: What type of patient should be diagnosed?
In summary, combined with the patient's symptoms, signs and auxiliary examination results, the final diagnosis is: (clinically confirmed) amyotrophic lateral sclerosis
.
Q8: How is ALS clinically treated^[3]?
ALS remains an incurable disease
.

However, there are many ways to improve the quality of life of patients, and early diagnosis and early treatment should be used to prolong survival as much as possible
.
(1) Riluzole: chemical name is 2-amino-6 (trifluoromethoxy)-benzothiazole, and its mechanism of action includes stabilizing the inactive state of voltage-gated sodium channels, inhibiting presynaptic glutamate release, activating postsynaptic glutamate receptors to promote glutamic acid uptake, etc
.
The usage is 50 mg orally
2 times a day.

Common adverse effects are fatigue and nausea, and some patients may have elevated alanine aminotransferase, and liver function
should be monitored.

When patients with advanced disease have already used an invasive ventilator to assist breathing, it is not recommended to continue taking it
.
(2) Other drugs: the free radical scavenger edalafen can delay the disease process
under certain conditions.
(3) In addition, comprehensive treatment such as nutritional management, respiratory support, and psychotherapy is also very important
.

small

knot

The early clinical manifestations of ALS are diverse, lacking specific biologically confirmatory indicators
.

A detailed medical history, a detailed physical examination, and a standardized neuroelectrophysiological examination play a key role in early diagnosis, and other auxiliary tests such as imaging have some value
in differential diagnosis.

In clinical work, early diagnosis and early treatment should be used to delay disease progression
.

References:

Cui Liying, Pu Chuanqiang, Fan Dongsheng, et al.
Guidelines for the diagnosis and treatment of amyotrophic lateral sclerosis in China[J].
Chinese Journal of Neurology,2012(07):531-533.

[2] Oskarsson B,Gendron T F,Staff N P.
Amyotrophic Lateral Sclerosis:An Update for 2018[J].
Mayo Clin Proc,2018,93(11）:1617-1628.

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Source of this articleMedical Neurology Channel This article author | LL This document reviews | Deng Caihong, Deputy Chief Physician
Responsible EditorMr.
Lu Li Xiang Yu

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