echemi logo
Product
  • Product
  • Supplier
  • Inquiry
    Home > Active Ingredient News > Study of Nervous System > The 40-year-old woman appeared "butterfly bone", the reason behind...clinical reasoning

    The 40-year-old woman appeared "butterfly bone", the reason behind...clinical reasoning

    • Last Update: 2021-06-17
    • Source: Internet
    • Author: User
    Search more information of high quality chemicals, good prices and reliable suppliers, visit www.echemi.com
    Have you mastered the diagnosis and differential diagnosis of pterygium? Recently, the clinical reasoning series of Neurology reported a case of a 40-year-old middle-aged woman with right shoulder weakness and dysphonia.
    Let’s take a look at the clinical reasoning process
    .

    Translation: Reflection Wuhen This article is published by Yimaitong authorized by the author, please do not reprint without authorization
    .

     A 140-year-old woman with right shoulder weakness for 1 year.
    .
    .
    The patient was a 40-year-old woman who presented to the doctor with “right shoulder weakness for 1 year”
    .

    One year ago, the patient gradually had difficulty raising his right arm over his head, and difficulty in taking out items from the rear seat of the car
    .

    About 3 months after the onset of muscle weakness, the patient began to experience intermittent pain and hoarseness in the right ear
    .

    Denies changes in vision, taste, or hearing, dysphagia, dyspnea, numbness, or other physical weakness
    .

    There is no previous shoulder trauma, and no history of neck surgery; no severe shoulder pain before the onset of symptoms; no family history of shoulder weakness or gait disturbance
    .

    Physical examination showed: normal level of consciousness; fluent spoken language, normal retelling, naming and understanding; hoarse voice, low pitch, but all words are intelligible, without nasal sounds; the right soft palate elevation is limited; the pharyngeal reflex exists; motor examination It shows that the right shoulder is drooping, the right trapezius muscle is atrophy, and the outer edge of the right scapula is raised at rest, and the shoulder is abducted and aggravated
    .

    The maximum forward bend of the right shoulder is 120 degrees, but it can be increased to 180 degrees when lying on the back
    .

    Examination of a single muscle group showed 4/5 weakness of left neck rotation, 5-/5 neck flexion and 4/5 weakness of right shoulder shrug; no obvious abnormalities were seen in sensation and deep tendon reflexes
    .

     Questions to ponder 1.
    Orientation diagnosis? 2What positioning inspection and diagnosis should be performed? Neurogenic pterygoid scapula can be attributed to long thoracic neuropathy, paraneuropathy, or the less common dorsal scapular neuropathy
    .

    The serratus anterior (long thoracic nerve) will cause the inner edge of the scapula to bulge, and the shoulder joint will be more prominent when flexed
    .

    Paraneuropathy can cause the outer edge of the scapula to bulge, and shoulder abduction and aggravation
    .

    Rhomboid muscle weakness (scapular dorsal nerve) is painful on the inner edge of the scapula, and the elbow is pushed back
    .

    The combination of sagging shoulders, trapezius atrophy, lateral pterygoid scapula (increased abduction), and ipsilateral sternocleidomastoid weakness (neck flexion and contralateral neck rotation) suggest the presence of accessory neuropathy
    .

    Nervous system examination also showed that voice changes are consistent with dysphonia; soft palate elevation disorders may indicate vagus nerve dysfunction; behind the ear pain may indicate glossopharyngeal nerve dysfunction or non-specific metastatic pain
    .

    In order to further evaluate the patient's right pterygoid scapula, the patient underwent an EMG examination
    .

    The results showed that the upper limb motor and sensory nerve conduction were normal; the needle electromyography showed the right trapezius muscle fibrillation potential and nerve reinnervation (large long motor unit, reduced recruitment); serratus anterior muscle, rhomboid muscle, infraspinatus muscle , Cervical paraspinal muscles and limb muscles are normal
    .

    Laryngoscopy showed paralysis of the vocal cords
    .

     Questions to ponder 1.
    What is the cause? 2.
    What further inspections should be performed? 3 Why are these symptoms caused? The electromyography results suggest right side paraneuropathy or C3-C4 radiculopathy
    .

    Preservation of sensory nerve action potentials usually indicates abnormalities near the dorsal root ganglion
    .

    However, the accessory nerve has no sensory branch, so it is difficult to distinguish preganglionic disease from postganglionic disease without cervical paraspinal muscle involvement
    .

    The enhanced MRI scan of the spine showed no stenosis of the upper cervical segment
    .

    For this reason, the clinical manifestations are consistent with multiple cranial neuropathy
    .

    Pterygoid scapula is caused by paraneuropathy, but the cause is still unclear
    .

    The most common cause of paraneuropathy is iatrogenic (i.
    e.
    , lymph node biopsy at the back of the neck)
    .

    The distal lesions involve the trapezius muscle, but the sternocleidomastoid muscle is not involved; the proximal and intracranial lesions involve the sternocleidomastoid and trapezius muscles, as shown in this example
    .

    Head MRI and neck MRI showed enhanced space occupation of the right jugular foramen, suggesting suspicious meningioma or schwannoma (Figure 1)
    .

    In addition, atrophy of the right trapezius and sternocleidomastoid muscles can also be seen on imaging
    .

     Figure 1 MRI results of the head
    .

    A and B: Coronal (A) and axial (B) T1 enhancement shows the right jugular foramen (1.
    7cm × 1.
    6cm × 2.
    3cm) lesions, showing a typical dumbbell-shaped appearance and obvious uniform enhancement ( Arrow)
    .

     4How to treat? The patient underwent craniotomy to remove the tumor originating from the right glossopharyngeal nerve
    .

    The tumor is compressing the vagus nerve and accessory nerve, but there is no infiltration
    .

    The pathology showed benign schwannomas with mixed features of schwannomas and neurofibroma (Figure 2)
    .

    Figure 2 Pathological analysis results of jugular foramen tumor
    .

    A: H&E staining shows spindle cells and collagen matrix (×100 times)
    .

    B: S100 immunostaining showed diffuse positive (×100 times)
    .

    C: CD34 staining showed partial positive (×100 times)
    .

     In the next 3 months, the patient's voice improved rapidly; in the next year, the patient's pterygoid scapula improved slowly and steadily
    .

    5What is jugular foramen syndrome? This case caused paraneuropathy due to tumor occupation, manifested as multiple cranial nerve dysfunction, called jugular foramen syndrome (Vernet syndrome)
    .

    The reminder from this case is that if iatrogenic proximal paraneuropathy is excluded, compressive or neoplastic lesions should be sought
    .

    The foramen of the jugular vein is divided into two parts by a fibrous septum: the smaller anteromedial part (nerve part) and the larger posterolateral part (venous part)
    .

    The nerve includes the petrosal sinus, glossopharyngeal nerve, vagus nerve, and accessory nerve, while the vein includes the sigmoid sinus-jugular bulb complex
    .

    The hypoglossal nerve tube is located closely adjacent to the posterior medial side of the jugular foramen
    .

    Lesions confined to the foramen of the jugular vein can cause paralysis of the glossopharyngeal, vagus and accessory nerves
    .

    The diagnosis of neoplastic jugular foramina syndrome can be difficult because the cranial nerve involvement gradually develops, and the order of appearance and the degree of damage to each cranial nerve vary, as in this case
    .

    Lesions involving the jugular foramen can also extend beyond this structure and involve the hypoglossal nerve tube
    .

    Collet-Sicard syndrome is a neurological disease characterized by paralysis of the glossopharyngeal, vagus, accessory and hypoglossal nerves
    .

    Villaret syndrome refers to Horner's syndrome in addition to the paralysis of the glossopharyngeal, vagus, accessory and hypoglossal nerves, indicating that the carotid sheath is involved
    .

    The causes of jugular foramen syndrome include primary tumors (meningioma, schwannoma, cholesteatoma), inflammation (meningitis and malignant otitis media), bone metastases, cancerous meningitis, and internal jugular vein thrombosis
    .

    In this case, the pathology showed a "mixed" benign schwannoma
    .

    The presence of the envelope, the axons surrounding the lesion, and the diffuse S100 immunoreactivity all support schwannomas, while the obvious collagen and the lack of Verocay bodies in the tumor indicate the possible histology of neurofibromas
    .

    Neurofibromas usually form nodular or polypoid lesions on the skin
    .

    Schwannomas most commonly involve the vestibular cochlear nerve, followed by the trigeminal nerve
    .

    Neurilemmoma can be distinguished from other primary intracranial tumors by certain imaging features
    .

    For this patient, the space occupied by the jugular foramen showed a dumbbell shape and obvious diffuse enhancement, which is a typical imaging feature of schwannoma
    .

    Meningioma usually shows dural tail sign on imaging
    .

    Parsonage–Turner syndrome (or neuromuscular atrophy) is a common non-iatrogenic cause of pterygoid scapula
    .

    In this syndrome, the trapezius muscle is involved in 20% of cases, and can sometimes be accompanied by dysphonia, mimicking partial Vernet syndrome
    .

    For this inflammatory uniphasic disease, muscle weakness usually precedes severe shoulder pain, and usually causes diffuse, patchy involvement of the long thoracic nerve, suprascapular nerve, axillary nerve, and anterior interosseous nerve
    .

     There are many differential diagnoses of pterygoid scapula, and detailed clinical examination and electromyography may be very useful
    .

    Although the cause of the tumor is rare, it must be considered in unilateral pterygoid scapula, especially if there is no history of trauma or medical history
    .

      Original Index: Mohammad Aladawi, Michael Punsoni and Ezequiel Piccione.
    Clinical Reasoning: Forty-Year-Old Woman With Scapular Winging and Dysphonia.
    Neurology published online May 12, 2021.
    DOI 10.
    1212/WNL.
    0000000000012179.
    This article is an English version of an article which is originally in the Chinese language on echemi.com and is provided for information purposes only. This website makes no representation or warranty of any kind, either expressed or implied, as to the accuracy, completeness ownership or reliability of the article or any translations thereof. If you have any concerns or complaints relating to the article, please send an email, providing a detailed description of the concern or complaint, to service@echemi.com. A staff member will contact you within 5 working days. Once verified, infringing content will be removed immediately.

    Contact Us

    The source of this page with content of products and services is from Internet, which doesn't represent ECHEMI's opinion. If you have any queries, please write to service@echemi.com. It will be replied within 5 days.

    Moreover, if you find any instances of plagiarism from the page, please send email to service@echemi.com with relevant evidence.