Home
Keys to Success in Medicine
Cardiovascular System
Respiratory System
Locomotor System
Endocrine and Metabolic System
Kidneys and Urinary System
Gastrointestinal Tract
Central Nervous System
Haematological System
Integumental System
Reproductive and Genital System
Recommended Reading
Forum
Links

amazon astore

ydr

aces for paces

Syringomyelia

Title               

Ñ    Syringomyelia

Definition

Ñ    Syringomyelia is characterised by the presence of a fluid filled cavity (syrinx) within the spinal cord.

Ñ    Syringobulbia refers to a syrinx in the brainstem.

Pathogenesis

Causes

Ñ    Arnold-Chiari malformation is often associated with it

Ñ    Arnold-Chiari malformation refers to aberrant cerebellar tissue that extends through the foramen magnum:

Herniation of the cerebellar tonsil

Other associations are:

Ñ    Bony anomalies of the foramen magnum

Ñ    Klippel-Feil syndrome

Ñ    Spina bifida

Ñ    Arachnoiditis

Ñ    Hydrocephalus

Ñ    Intrinsic cord tumours such as glioma and ependymoma may be followed by syrinx formation

Pathological Process

Ñ    Anatomical abnormality at the foramen magnum

Ñ    Allows pulsatile CSF pressure waves to be transmitted to the tissues of the brain stem and spinal cord

Ñ    This results in formation of a cavity (syrinx)

Ñ    The syrinx is in continuity with the central canal of the spinal cord

Ñ    The expanding syrinx destroys the following:

Syringomyelia

Ñ    Anterior horn cells

Ñ    Lateral corticospinal tracts

Ñ    Spinothalamic neurones

Syringobulbia

Ñ    5^th nerve nucleus

Ñ    9^th, 10^th, 11^th, 12^th nerve nuclei

Ñ    Sympathetic system

Ñ    Vestibular system

Clinical Features

History

Ñ    Symptoms develop between 20-30 years

CNS

Ñ    Upper limb pain aggravated by coughing or exertion

Ñ    Difficulty in walking:

Later

IS

Ñ    Painless burns

Ñ    Trophic changes

Examination

IS

Ñ    Burns

Ñ    Trophic changes

CNS

Syringomyelia

Ñ    Weakness and wasting of the small muscles of the hand and the forearm muscles

Ñ    Loss of upper limb reflexes

Ñ    Dissociate sensory loss in upper limbs and upper chest (cape distribution):

Loss of pain and temperature

Intact light touch and proprioception

Ñ    Spastic paraparesis

Brainstem signs in syringobulbia

Ñ    Horner’s syndrome

Ñ    Nystagmus

Ñ    Facial sensory loss

Ñ    Hearing loss

Ñ    Bulbar palsy

LMS

Ñ    Charcot’s joints

Investigations

Imaging

MRI

Ñ    Demonstrates syrinx clearly

Management

Control

Surgery

Ñ    Surgical decompression may be attempted

Ñ    Decompression of foramen magnum

Ñ    Syringoperitoneal shunting

Prognosis

Ñ    Slowly progressive

[ Back ] [ Up ] [ Next ]

[Up]