Abadie's sign of tabesdorsalis may be elicited during clinical examination. Pinching of, or the application of firm pressure to, the Achilles tendon does not result in pain in tabesdorsalis. This is because the sense of deep pain has been abolished.It is named for Joseph Louis Irenée Jean Abadie, a French neurologist. Abadie's sign of exophthalmic goiter is Spasm of the LevatorPalpebraeSuperioris muscle with retraction of the upper lid (so that sclerais visible above cornea) seen in Graves-Basedow disease which, together with exophthalmos causes the bulging eyes appearance.It is named for Jean Marie Charles Abadie.
SMA is traditionally classified by age of onset and severity, however some experts classify by functional levels (non-sitters, sitters, and walkers) as which correlate better with clinical care needs than the traditional classification. [Wang: 2007] Clinical characteristics often overlap between types.Type I SMA (Werdnig-Hoffman ) presents near birth; these children never learn to sit or walk and have severe respiratory and swallowing problems, including difficulty handling oral secretions, and a significantly shortened life span. Risk of death in infancy is high, and most survivors are ventilator dependent by two years of age. Other features include poor head control, a bell-shaped chest, weak cry and cough, tongue atrophy and fasciculation, and paradoxical breathing.Type II SMA presents later in the first year of life or up to about two years of age. These children usually learn to sit. They often have respiratory and swallowing problems, including difficulty gaining weight due to bulbar muscle weakness, weak cough and night-time hypoventilation. Swallowing problems and difficulties opening the jaw widely have been shown to contribute to malnutrition in children with SMA II. [Messina: 2008] A tremor may be noted in these children. Joint contractures and scoliosis develop over time in nearly all affected children, and warranting proactive intervention.Type III SMA (Kugelberg-Welander) presents later in childhood or adolescence and these children usually sit and walk (although some may lose this ability over time) and have fewer problems with respiratory function and swallowing. Scoliosis and contractures as well as joint pain are often noted in older children.Infants with type 0 SMA present before birth, with decreased fetal movement noted around 30 weeks of age. They are hypotonic at birth with swallowing and breathing problems and, in some infants, arthrogryposis.Type IV SMA presents in adults.
ALS is often called Lou Gehrig's disease in North America, after the New York Yankees baseball player who was diagnosed with the disease in 1939. The disorder is characterized by rapidly progressiveweakness, muscle atrophy and fasciculations, spasticity, dysarthria, dysphagia, and respiratory compromise. Sensory function generally is spared, as is autonomic and oculomotor activity. ALS is a progressive, fatal, neurodegenerative disease with most affected patients dying of respiratory compromise and pneumonia after 2 to 3 years; although some perish within a year from the onset of symptoms, and occasional individuals have a more indolent course and survive for many years.
Spinal cord syndromes
In the Name of God, Most Gracious, Most Merciful
SPINAL CORDSYNDROMES - Dr. Mohammed Sadiq Azam II yr. Postgraduate MD Internal Medicine Deccan College of Medical Sciences
COMPLETE CORD TRANSECTION• All ascending tracts frombelow and descending tractsfrom above are interrupted.• Affects motor, sensory andautonomic functions.
COMPLETE CORD TRANSECTIONSENSORY: All sensations are affected. Pin prick test is very valuable. Sensory level is usually 2 segments below the level of lesion. Segmental paraesthesia occur at the level of lesion.
COMPLETE CORD TRANSECTIONMOTOR: Paraplegia due to corticospinal tract involvement. First spinal shock-followed by hypertonic hyperreflexic paraplegia. Loss of abdominal and cremastric reflexes. At the level of lesion LMN signs occur.
COMPLETE CORD TRANSECTIONAUTONOMIC: Urinary retention and constipation. Anhidrosis, trophic skin changes, vasomotor instability below the level of lesion. Sexual dysfunction can occur.
BROWN SEQUARD SYNDROME = Hemi-section of the spinal cord Caused by extramedullary lesions Usually caused by penetrating trauma or tumour.
BROWN SEQUARD SYNDROMESENSORY: Ipsilateral loss of proprioception due to posterior column involvement. Contralateral loss of pain and temperature due to involvement of lateral spinothalamic tract.
BROWN SEQUARD SYNDROMEMOTOR: Ipsilateral spastic weakness due to descending corticospinal tract involvement LMN signs at the level of lesion.
CENTRAL CORD SYNDROME Commonest cause is Syringomyelia. Other causes: ◦ Hyperextension injuries of neck ◦ Intramedullary tumours ◦ Trauma ◦ Associated with Arnold Chiari type 1 and 2, Dandy walker malformation
CENTRAL CORD SYNDROMESENSORY: Pain and temperature are affected. Touch and proprioception are preserved. Dissociative anaesthesia. Shawl like (= Cape like) distribution of sensory loss.MOTOR: Upper limb weakness > Lower limb
SYRINGOMYELIAOTHER FEATURES : Horner’s syndrome Kyphoscoliosis Sacral sparing Neuropathic arthropathy of shoulder and elbow joint Prognosis is fair.
POSTERIOR CORD SYNDROME Commonest causes include diabetes mellitus & neurosyphilis. Usually occurs 10 to 20 yrs after disease onset.
POSTERIOR CORD SYNDROMESENSORY : Impaired position and vibration sense in LL Tactile and postural hallucinations can occur. Numbness or paresthesia are frequent complaints.. Sensory ataxia. Positive rhomberg sign. Positive sink sign Positive lhermittes sign.
POSTERIOR CORD SYNDROMESENSORY (contd): Abadie’s sign (of tabes dorsalis) positive Urinary incontinence Absent knee and ankle jerk (Areflexia, Hypotonia) Charcot’s joint Miotic and irregular pupil not reacting to light Argyl Robertson Pupil
POSTERIO LATERAL COLUMN DISEASEFEATURES : Paresthesia in feet Loss of proprioception and vibration in legs Sensory ataxia Positive Rhomberg sign Bladder atony Corticospinal tract involvement: ◦ Spasticity ◦ Hyperreflexia ◦ Bilateral Babinski sign - Positive
POSTERIO LATERAL COLUMN DISEASE AIDS: ◦ Associated dementia and spastic bladder is present HTLV associated myelopathy: ◦ Slowly progressive paraparesis ◦ Increase in CSF IgG with antibodies to HTLV 1
ANTERIOR CORD SYNDROME Due to acute disc herniation or ischemia from anterior spinal artery occlusion. Usually caused by hyperflexion injuries Area supplied by anterior spinal artery is affected
ANTERIOR CORD SYNDROME Sudden onset of paralysis (quadriparesis/paraparesis) below the level of lesion. Pain and temperature loss. Dorsal column is preserved. Prognosis is poor.
ANTERIOR SPINAL ARTERY SYNDROME Commonest of the vascular syndromes of the cord. Spinal cord infarction usually occurs in T1 to T4 segment & L1. Occurs due to aortic dissection, atherosclerosis of aorta, SLE, AIDS, AV malformation Rarely due to dissection of the anterior spinal artery or systemic arteritis. Syphilitic arteritis is now rare. Conus medullaris is frequently involved. Neck pain of sudden onset is a common feature. Also called as “Beck’s syndrome”.
ANTERIOR SPINAL ARTERY SYNDROMESENSORY : Loss of pain and temperature. Preservation of position and vibration.MOTOR : Sudden onset flaccid and areflexic paraplegia.AUTONOMIC : Urinary incontinence +
POSTERIOR SPINAL ARTERY SYNDROME UNCOMMON Loss of proprioception and vibratory sense. Pain and temperature is preserved. Absence of motor deficit.
ANTERIOR HORN CELL SYNDROMES CAUSED BY SPINAL MUSCULAR ATROPHY ◦ Spinal muscular atrophy (SMA) is an autosomal recessive disorder that causes decreased survival of the anterior horn cells – motor neurons – that innervate voluntary muscles, resulting in progressive muscle atrophy and weakness. ◦ Types I to IV ◦ Eponyms: Werdnig-Hoffman disease, Kugelberg-Welander disease, SMA, Anterior horn cell disease
ANTERIOR HORN CELL SYNDROMESMOTOR : Weakness, atrophy and fasciculations. Hypotonia, depressed reflexes. Muscles of trunk and extremities are affected. Sensory system is not affected.
ANTERIOR HORN CELL & PYRAMIDAL TRACT SYNDROME Occurs in amytrophic lateral sclerosis (ALS). ◦ Also called Lou Gehrigs disease. ◦ A form of Motor Neuron Disease caused by the degeneration of upper and lower neurons, located in the ventral horn of the spinal cord and the cortical neurons that provide their efferent input. Affects the anterior horn cells and corticospinal tract. Both LMN and UMN signs occur.
ANTERIOR HORN CELL & PYRAMIDAL TRACT SYNDROMEMOTOR : Ant horn cell related: ◦ Paresis, Atrophy and Fasciculations. Corticospinal tract related: ◦ Paresis, Spasticity and Extensor plantar response.
ANTERIOR HORN CELL & PYRAMIDAL TRACT SYNDROME It is usually unilateral with muscle weakness + Reflexes are often exaggerated. Bulbar and pseudo bulbar involvement occurs. Sensory system is not affected. Superficial reflex - Abdominal reflex is preserved.
CONUS MEDULLARIS SYNDROME CM: Lies opposite to vertebral bodies of T12 and L1. Contributes to 25% of spinal cord injuries. Caused by flexion distraction injuries and burst fractures. Both UMN and LMN deficits occur. Development of neurogenic bladder.
CAUDA EQUINA SYNDROME CE: Begins at L2 disk space distal to conus medullaris. CE syndrome occurs due to: ◦ Acute disk herniation ◦ Epidural haematoma ◦ Tumour
CAUDA EQUINA SYNDROMEMOTOR : Flaccid lower extremities. Knee and ankle jerk absent.SENSORY : Asymmetrical sensory loss Saddle anaesthesia Loss of sensation around perineum, anus, genitals.
CAUDA EQUINA SYNDROMEAUTONOMIC: Loss of bladder and bowel function. Urinary retention.
DDx: CONUS vs CAUDAFEATURE CONUS MEDULARIS CAUDA EQUINAPRESENTATION Sudden & Bilateral Gradual & UnilateralREFLEXES Knee present, Ankle – Knee & Ankle – (If the epiconus is involved, patellar reflex Bulbocavernosus reflex maybe absent but is absent in low CE bulbocavernosus is (sacral) lesions spared)RADICULAR PAIN Less severe More severeLOW BACK ACHE More Less Ref: http://www.emedicine.com/neuro/topic667.htm
FEATURE CONUS MEDULARIS CAUDA EQUINASENSORY Numbness tends to be Numbness tends to be moreSYMPTOMS more localized to localized to saddle area; perianal area; asymmetrical, maybe symmetrical and unilateral; no sensory bilateral; sensory dissociation; loss of sensation dissociation occurs. in specific dermatomes in lower extremities with numbness and Sensory loss of pin paresthesia; possible numbness prick & temperature in pubic area, including glans sensations (Tactile penis or clitoris. sensation is spared.)Ref: http://www.emedicine.com/neuro/topic667.htm
FEATURE CONUS MEDULARIS CAUDA EQUINAMOTOR Typically symmetric, Asymmetric areflexic paraplegiaSYMPTOMS distal paresis of lower that is more marked; limbs that is less fasciculations rare; atrophy more common . marked; fasciculations may be present.IMPOTENCE Frequent Less frequent; ED is common erectile dysfunction that includes inability to have erection, inability to maintain erection, lack of sensation in pubic area (including glans penis or clitoris), and inability to ejaculate.Ref: http://www.emedicine.com/neuro/topic667.htm
FEATURE CONUS MEDULARIS CAUDA EQUINASPHINCTER Urinary retention and Urinary retentionDYSFUNCTION atonic anal sphincter cause overflow urinary Tends to present late in incontinence and fecal course of disease incontinence Tend to present early in course of disease.EMG Mostly normal lower Multiple root level extremity with external involvement; sphincters may anal sphincter invlmnt also be involved.OUTCOME Less favourable More FavourableRef: http://www.emedicine.com/neuro/topic667.htm