More Related Content
Similar to Neuro-hour-syringomyelia.pptx
Similar to Neuro-hour-syringomyelia.pptx (20)
Recently uploaded
Recently uploaded (20)
Neuro-hour-syringomyelia.pptx
- 2. Objectives 01 Case 02 Localization 03 Course in the ward 05 Discussion 06 Concept map 07 Outline Primary Diagnosis and differentials 04 Take home message 08
- 3. Objectives • Discuss the pathophysiology of syringomyelia and syringobulbia • Discuss the localization of lesions based on history and physical examination •
- 4. Case • J.C • 44/F • Filipino • Roman Catholic • Teacher • South Cotabato
- 6. HPI 2009 • Patient was diagnosed with ependymoma at C3 – T1 and underwent Laminectomy at C1 and T3 • Residual deficits include numbness of the upper chest, upper arm and neck area
- 8. HPI 2 months PTA • Patient had hoarseness of voice associated with residual deficits of numbness in the neck, upper arms and upper chest area • Consult with ENT • Laryngoscopy done but unremarkable • Consult with Neuro-surgeon
- 9. HPI 1 day PTA • Had progression of numbness in the same areas as claimed with associated hoarseness of voice • Advised admission
- 10. Neuro PE Cerebral: oriented to time, place and person intact immediate, recent and remote memory follows commands Cerebellar: (-) rhomberg’s test (-) dysmetria (-) dysdiadochokinesia
- 11. Neuro PE Cranial Nerves I – not assessed II, III – no visual field defects, PERLA, isocoric III, IV, VI – intact EOM V, VII – no facial asymmetry VIII – able to hear finger rub IX, X – (+) gag reflex XI – able to shrug shoulders XII – no tongue deviation, no tongue atrophy
- 12. Neuro PE Motor: Right Left Arm flexion (C5, C6) 5/5 5/5 Arm Extension (C5, C6) 5/5 5/5 Wrist extension (C6, C7, C8) 4/5 5/5 Finger abduction (C8, T1) 2/5 4/5 Hip flexion (L2, L3, L4) 5/5 5/5 Hip extension (S1) 5/5 5/5
- 13. Neuro PE Sensory – fine touch Level Right Left Neck (C2,C3) 20% 20% Shoulder (C4) 20% 20% Chest (T2,T3) 80% 80% Nipple Level to umbilicus (T4-T10) 100% 100% Below umbilicus to inguinal area (T11-L1) 100% 100% Upper and lower leg (L2-S1) 100% 50%
- 14. Neuro PE Sensory – Pain and temperature Level Right Left Neck (C2,C3) 50% 50% Shoulder (C4) 50% 50% Chest (T2,T3) 100% 100% Nipple Level to umbilicus (T4-T10) 100% 100% Below umbilicus to inguinal area (T11-L1) 100% 100% Upper and lower leg (L2-S1) 100% 100%
- 15. Neuro PE Sensory – vibration Level Right Left Neck (C2,C3) Intact Intact Shoulder (C4) Intact Intact Chest (T2,T3) Intact Intact Nipple Level to umbilicus (T4-T10) Intact Intact Below umbilicus to inguinal area (T11-L1) Intact Intact Upper and lower leg (L2-S1) Intact Intact
- 16. Neuro PE Sensory – position Level Right Left Neck (C2,C3) Intact Intact Shoulder (C4) Intact Intact Chest (T2,T3) Intact Intact Nipple Level to umbilicus (T4-T10) Intact Intact Below umbilicus to inguinal area (T11-L1) Intact Intact Upper and lower leg (L2-S1) Intact Intact
- 17. Neuro PE Reflexes Right Left Biceps (C5, C6) 0 0 Triceps (C6, C7) 0 0 Patellar (L2, L3, L4) 2+ 1+ Ankle (S1) 2+ 1+ Babinski (-) (-)
- 18. Localization • Localization starts with the question… • Supratentorial vs infratentorial • Upper motor vs lower motor neurons • If spinal cord involvement – intramedullary vs extramedullary intradural vs extramedullary extradural
- 19. Intramedullary vs extramedullary System Features Extramedullary Intramedullary History Onset Asymmetry Symmetrical Pain Local or vertebral (extradural) Radicular (intradural) Funicular or tract pain MOTOR UMN signs Early Late LMN sgns Segmental Diffuse Sensory Sensory involvement Ascending (sacral involvement) Descending (sacral sparing) Dissociated sensory loss Absent Present
- 20. Upper vs Lower motor neuron lesion by PE UMN LMN Type of paralysis Spastic Paresis Flaccid paresis Atrophy No atrophy Severe atrophy Deep tendon reflex Increase Absent Pathologic reflex Babinski Absent Superficial reflex Absent Present Fasciculation Absent Present
- 21. Discussion Spinal cord level Corresponding vertebral body Upper cervical Same as cord level Lower cervical 1 level higher Upper thoracic 2 levels higher Lower thoracic 2-3 levels higher Lumbar T10-T12 Sacral T12-L1
- 22. Localization Pain and temperature sensation: • Decreased at Neck (C2,C3) • Shoulder (C4)
- 23. Localization Pain and temperature sensation: • Decreased at Neck (C2,C3) • Shoulder (C4)
- 24. Localization Motor: • Wrist extension (C6, C7, C8) – 4/5 in the right • Finger abduction (C8, T1) – 2/5 in the right
- 26. TB, cervical spine C2-T1 Differentials Cervical cord adhesions Ependymom a, recurrence
- 28. Laboratories CBC WBC 10.7 59/31/8/1/0 Hgb 14.5 Hct 44.6 Plt 265 Creatinine 0.38 SGPT 33 TSH 1.5 FT4 13.2 FT3 4.7
- 29. Short Course ● Preop evaluation ● Medically cleared cardio and pulmo- wise ● Had episoodes of anxiety – relieved with reassurance and O2 supplementation Day 1-6 Day 8 – 12 ● ICU care ● Treated for CAP HR ● Still with numbess on upper back, neck and upper arms ● Weaned off MV Day 7 ● S/P cervical adhesiolysis C2-C6 ● ICU post op
- 30. Fine Touch (post-op) Level Right Left Neck (C2,C3) 30% 30% Shoulder (C4) 30% 30% Chest (T2,T3) 80% 80% Nipple Level to umbilicus (T4- T10) 100% 100% Below umbilicus to inguinal area (T11-L1) 100% 100% Upper and lower leg (L2-S1) 100% 50%
- 31. Short Course ● Back to wards ● Antibiotics completed Day 13-16 Discharged
- 32. Discussion • The priority in neurologic examination is to identify the region of the nervous system that is likely responsible for the symptoms • Once the question, “where is the lesion?” is answered, then the question “what is the lesion?” can be addressed
- 33. Discussion • The presence of a horizontally defined level below which sensory, motor, and autonomic function is impaired is a hallmark of a lesion of the spinal cord
- 34. Ependymoma • Tumors derived from ependymal cells that line the ventricular surface • More common in children • Arise from the wall of the fourth ventricle in the posterior fossa • They occur more commonly in the spine • Can be completely resected are potentially curable
- 35. SYRINGOMYELIA • Syringomyelia is a developmental cavity of the cervical cord that may enlarge and produce progressive myelopathy or may remain asymptomatic. • More than half of all cases are associated with Chiari type 1 malformations in which the cerebellar tonsils protrude through the foramen magnum and into the cervical spinal canal. • Acquired cavitation of the cord in areas of necrosis are also termed syrinx cavities; these follow trauma, myelitis, necrotic spinal cord tumors, and chronic arachnoiditis due to tuberculosis and other etiologies.
- 36. SYRINGOMYELIA • MRI accurately identifies developmental and acquired syrinx cavities and their associated spinal cord enlargement • Syrinx cavities secondary to trauma or infection, if symptomatic, are treated with a decompression and drainage procedure in which a small shunt is inserted between the cavity and subarachnoid space.
- 37. Synringobulbia • neurological disorder characterized by a fluid-filled cavity (syrinx) within the spinal cord that extends to involve the brainstem (medulla) • occurs as a slit-like gap within the lower brainstem that may affect one or more of the cranial nerves, causing facial palsies of various kinds • This disorder is intimately associated with syringomyelia, in which the syrinx is limited to the spinal cord, and to the Chiari I malformation. • Treatment of syringobulbia is almost invariably surgical and consists of efforts to reroute the flow of cerebrospinal fluid by the use of diversion tubes or shunts
- 38. Concept Map 44/F Hypertensive Ependymoma (2009) s/p Laminectomy C3 and T1 Hoarseness of Voice Numbness of neck, upper arms and upper chest Excessive tissue repair Blockage of CSF flow Compression of spinal cord at central canal Destruction of the adjacent gray and white matter Compression of medulla Loss of pain and temperature sensation Extension into medulla Adhesions Loss of communication between peripheral nerve and CNS Adhesiolysis Pregabalin
- 39. Take home message • Neuroimaging procedures and laboratory tests, which, while useful, do not substitute for an adequate history and examination • Treatment of syringomyelia and syringobulbia is largely unsatisfactory, but usually involves surgical decompression
Editor's Notes
- MRI last 2019 long segment intramedullary lesion from the medulla to T5 level
- The relationship between spinal cord segments and the corresponding vertebral bodies is shown in Table
- Sensory loss below this level is the result of damage to the spinothalamic tract on the opposite side, one to two segments higher in the case of a unilateral spinal cord lesion, and at the level of a bilateral lesion. The discrepancy in the level of a unilateral lesion is the result of the course of the second-order sensory fibers, which originate in the dorsal horn, and ascend for one or two levels as they cross anterior to the central canal to join the opposite spinothalamic tract. NOTES: The first order neurones arise from the sensory receptors in the periphery. They enter the spinal cord, ascend 1-2 vertebral levels, and synapse at the tip of the dorsal horn – an area known as the substantia gelatinosa. The second order neurones carry the sensory information from the substantia gelatinosa to the thalamus. After synapsing with the first order neurones, these fibres decussate within the spinal cord, and then form anterior and lateral spinothalamic tracts The third order neurones carry the sensory signals from the thalamus to the ipsilateral primary sensory cortex of the brain. They ascend from the ventral posterolateral nucleus of the thalamus, travel through the internal capsule and terminate at the sensory cortex.
- Sensory loss below this level is the result of damage to the spinothalamic tract on the opposite side, one to two segments higher in the case of a unilateral spinal cord lesion, and at the level of a bilateral lesion. The discrepancy in the level of a unilateral lesion is the result of the course of the second-order sensory fibers, which originate in the dorsal horn, and ascend for one or two levels as they cross anterior to the central canal to join the opposite spinothalamic tract. NOTES: The first order neurones arise from the sensory receptors in the periphery. They enter the spinal cord, ascend 1-2 vertebral levels, and synapse at the tip of the dorsal horn – an area known as the substantia gelatinosa. The second order neurones carry the sensory information from the substantia gelatinosa to the thalamus. After synapsing with the first order neurones, these fibres decussate within the spinal cord, and then form anterior and lateral spinothalamic tracts The third order neurones carry the sensory signals from the thalamus to the ipsilateral primary sensory cortex of the brain. They ascend from the ventral posterolateral nucleus of the thalamus, travel through the internal capsule and terminate at the sensory cortex.
- CTS Motor cortex (area 4) > Corona Radiata > internal capsule > midbrain > medullary pyramids (decussation) 90% lateral corticospinal tract, 10% will not decussate and form the anterior corticospinal tract > anterior horn Corticobulbar Tracts synapsing in the brainstem with motor nuclei of the cranial nerves are termed corticobulbar. The corticobulbar tract is composed of the upper motor neurons of the cranial nerves. The muscles of the face, head and neck are controlled by the corticobulbar system,
- My primary impression is syringobulbia since we are presented with a 44 yr old female patient who had a previous history of ependymoma who underwent laminectomy at C2 and T1. Due to the trauma, there is accumulation of fluid filled syrinx in the spinal cord, which causes compression and damage. Extension of the syrinx into the medulla, syringobulbia, causes palatal or vocal cord paralysis, dysarthria, horizontal or vertical nystagmus, episodic dizziness or vertigo, and tongue weakness with atrophy
- The first differential is Cervical cord adhesions since we are presented with a patient who underwent laminectomy at c2 –T1. This causes formation of scar tissue leading to adhesions which will obstruction of CSF flow and compression of spinal cord structures TB of the cervical spine is considered since Tb is endemic in the Philippines and is one of the most common causes of spinal cord compression in developing countries.
- No significant interval change in the long segment intramedullary lesion from the medulla to T5 level when compared to the study last 2019 C4-C5, C5-C6, C6-T1 mild posterior disc protrusions
- The priority in neurologic examination is to identify the region of the nervous system that is likely responsible for the symptoms. Can the disorder be mapped to one specific location, is it multifocal, or is a diffuse process present? Are the symptoms restricted to the nervous system? Or do they arise in the context of a systemic illness? The proper approach begins with the patient and focuses the clinical problem first in anatomic and then in pathophysiologic terms
- The relationship between spinal cord segments and the corresponding vertebral bodies is shown in Table
- Tumors derived from ependymal cells that line the ventricular surface More common in children Arise from the wall of the fourth ventricle in the posterior fossa They occur more commonly in the spine Can be completely resected are potentially curable Partially resected ependymomas will recur and require irradiation. chemotherapy has limited efficacy
- Muscle wasting in the lower neck, shoulders, arms, and hands with asymmetric or absent reflexes in the arms reflects expansion of the cavity in the gray matter of the cord
- Extension of the syrinx into the medulla, syringobulbia, causes palatal or vocal cord paralysis, dysarthria, horizontal or vertical nystagmus, episodic dizziness or vertigo, and tongue weakness with atrophy
- although isolated cases of syringobulbia have been documented.