1. Spinal chord

2. Spinal cord & Spinal Nerves
The spinal cord (45cm long) is part of the central nervous system and is connected to the brain
Extends from the foramen magnum to the first lumbar vertebra (L1).
The spinal cord is the main pathway for information connecting the brain and peripheral nervous system.
31 pairs of spinal nerves

3. Spinal Cord Protection
Bony Canal- vertebral canal
Connective tissue- meninges
Cushion of cerebrospinal fluid (CSF)- CSF acts as a shock absorber

4. Meninges The meninges surround, protect, & stabilize the CNS
Consist of 3 layers
DURAMATER
ARACHNOID
PIAMATER

5. Meninges Epidural space- a space between bony canal & dura mater
Subdural space- A space between the dura mater & arachnoid mater- contains interstitial fluiid
Subarachnoid space- contains CSF fluid

7. Internal Anatomy of Spinal cord
Gray & white mater
H- shaped gray matter (central zone): dorsal & ventral horns
White matter (outer zone): - arrange in columns

8. Spinal Cord levels
The greatest amount of grey matter (cells) is largest in the
spinal segments of the cervical and lumbosacral enlargements

9. White mater (regions) Anterior white column Posterior white column
Lateral white column

10. Tracts of Spinal Cord (Sensory & motor)

11. Functions of spinal cord
Spinal cord reflexes: A reflex is a fast , involuntary, unplanned sequence of actions occur in response to a particular stimulus
Integration of information
highway for upward and downward travel of sensory and motor information

12. Classical spinal cord syndromes
Lesioned structures
Causes
Complete section
Mielitis
Trauma
Hemisection
Brown-Séquard
Central structures
Syringomyelia
Hydromyelia
Tumor
Posterior columns and horns
Tabes dorsalis
Posterior columns and lateral columns
B12 deficiency

13. Classical spinal cord syndromes
Lesioned structures
Causes
Dorsal columns, pyramidal tracts, spinocerebellar tract
Spinocerebellar degeneration
Pyramidal tracts, anterior horns
ALS
Anterior horn
Spinal muscle atrophy,
poliomyelitis
Pyramidal direct and crossed tracts
Familial spastic paraparesis
Dorsal 1/3 of the spine
Occlusion of the posterior spinal arteries
Anterior 2/3 of the spine
Anterior spinal artery occlusion

14. Traumatic Spinal Cord Disease
10,000 new spinal cord injuries per year
MVA, sports injuries the most common
Victims under 30 yrs old, male>>females
Fx/dislocation of vertabrae most likely to occur at:
C5,6
T12, L1
C1,2

15. Tumors Metastatic or primary Extramedullary Extradural - most common
Bony - breast, prostate
Intradural - very rare
Meninges - meningioma
Nerve root - schwannoma
Intramedullary - very rare
Metastatic
Primary - astrocytoma or ependymoma

16. Centromedular formations

17. Intramedular tumors
Anterolateral column compression lead to sensory (thermoalgesic) loss that extends lower as the tumor grows

18. Lateral compression
Sensory disturbance appears first in the sacrate dermatoma; as the tumor grows, the sensory problems ascend upwards to the level of the lesion

19. Transverse myelitis Inflammation of the spinal cord
Post-infectious
Post-vaccinial
Multiple sclerosis
Pain at level of lesion may preceed onset of weakness/sensory change/b&b disturbance
Spinal tap may help with diagnosis

21. Infections Involving the Spinal Cord
Polio
only the anterior horn cells are infected
Tabes dorsalis
dorsal root ganglia and dorsal columns are involved
tertiary syphillis
sensory ataxia, “lightening pains”
HIV myelopathy
mimics B12 deficiency
HTLV-1 myelopathy -
tropical spastic paraparesis

22. Multiple Sclerosis Demyelination is the underlying pathology
Cord disease can be presenting feature of MS or occur at any time during the course of the disease
Lesion can be at any level of the cord
Patchy
Transverse
Devic’s syndrome or myelitis optica
Transverse myelitis with optic neuritis

23. Vascular Diseases of the Spinal Cord
Infarcts
Anterior spinal artery infarct
from atherosclerosis, during surgery in which the aorta is clamped, dissecting aortic aneurysm
less often, chronic meningitis or following trauma
posterior columns preserved (JPS, vib)
weakness (CST) and pain/temperature loss (spinothalamic tracts)
Artery of Adamkiewicz at T10-11
Watershed area
upper thoracic

24. Vascular Diseases of the Spinal Cord, cont
Arteriovenous malformation (AVM) and venous angiomas
Both occur in primarily the thoracic cord
May present either acutely, subacutely or chronically (act as a compressive lesion)
Can cause recurrent symptoms
If they bleed
Associated with pain and bloody CSF
Notoriously difficult to diagnose
Hematoma - trauma, occasionally tumor

25. Other Diseases of the Spinal Cord
Hereditary spastic paraparesis
Usually autosomal dominant
Infectious process of the vertabrae
TB, bacterial
Herniated disc with cord compression
Most herniated discs are lateral and only compress a nerve root
Degenerative disease of the vertabrae
Cervical spondylosis with a myelopathy
Spinal stenosis

26. Brown Sequard Syndrome
Cord hemisection
Trauma or tumor
Dissociated sensory loss
loss of pain and temperature contralateral to lesion, one or 2 levels below
crossing of spinothalamic tracts 1-2 segments above where they enter
loss of vibration/proprioception ipsilateral to the lesion
these pathways cross at the level of the brainstem
Weakness and UMN findings ipsilateral to lesion

27. Syringomyelia Fluid filled cavitation in the center of the cord
Cervical cord most common site
Loss of pain and temperature related to the crossing fibers occurs early
cape like sensory loss
Weakness of muscles in arms with atrophy and hyporeflexia (AHC)
Later - CST involvement with brisk reflexes in the legs, spasticity, and weakness
May occur as a late sequelae to trauma
Can see in association with Arnold Chiari malformation

28. Syringomyelia

29. Conus Medullaris vs. Cauda Equina Lesion
Finding Conus CE
Motor Symmetric Asymmetric
Sensory loss Saddle Saddle
Pain Uncommon Common
Reflexes Increased/ Decreased
decreased
Bowel/bladder Common possible

30. B12 Deficiency Subacute combined degeneration of the cord
malabsorption of B12 secondary to pernicious anemia or surgery
insufficient dietary intake - vegan
Posterior columns and CST involvement with a superimposed peripheral neuropathy

31. Friedreich’s disease

32. Degeneration of the long spinal pathways (both ascending and descending) and the peripheral nerve fibers

33. Clinical presentation
Gait abnormalities
Ataxia
Speech disorders
Nistagmus
Hyporeflexia
Trophic changes
High plantar arches (pes cavus deformity of the foot)
Sensory disturbance
Babinski sign

34. Clinical presentation
Symptoms typically begin sometime between the ages of 5 to 15 years,
scoliosis
Diabetes (about 20% of people with Friedreich's ataxia develop carbohydrate intolerance and 10% develop diabetes mellitus)
Heart disorders (e.g., atrial fibrillation, and resultant tachycardia (fast heart rate) and hypertrophic cardiomyopathy )
These symptoms are slow and progressive. Long-term observation shows that many patients reach a plateau in symptoms in the patient's early adulthood.

36. Genetics Autosomal recesive disorder
Mutation of frataxin gene (9q13-q21)
Long arm of cromosome 9
Prevalence 1 :
Genetic counselling!

37. Treatment Physical therapy Nerve and muscle trophic drugs
Treatment of complications
Treatment of heart disorders

38. Lateral Amiotrophic Sclerosis

39. Lateral Amiotrophic Sclerosis (ALS)
Degenerative disease of the central nervous system
Degeneration and death of motor neurons, both in the cortex and in the spine
Mean survival time is 3 to 4 years from diagnosis
USA – “Lou Gehrig’s disease”

41. ALS – clinical picture progressing motor deficit
Signs related both to upper and to lower motor neuron damage
starting age is around 60 years (20-80 years)
Involvement of any part of the CNS
Either as UMN damage or as LMN damage

43. SLA – clinical picture Muscle weakness Atrophy Stiffness Cramps
In 60-85% of pts starts in the limbs and in % starts with bulbar signs
Atrophy
Stiffness
Cramps
Fasciculations;
Highly suggestive for ALS if largelly distributed and accompanied by UMN damage siogns

44. SLA – clinical picture
Respiratory Insufficiency is seldom present from the start
Dispnea, asteny, weak cough, predisposition for developping pneumonia ant athelectasies
With time, LMN signs may overcome all of the UMN signs
Brainstem damage signs
disphagia
disarthrie
sialorrhea (reduction of swallowing)
laringospasm
Lately in the course of disease oculomotor problems

45. Criteriile El Escorial revăzute pentru diagnosticul sclerozei laterale amiotrofice
Criteriul de diagnostic
Semne necesare
SLA sigură
Semne ale NMC şi ale NMP în cel puţin 3 din 6 regiuni
SLA familială sigură
Semne de afectare ale NMC şi NMP în o regiune (plus identificarea mutaţiei asociate prin procedee de laborator)
SLA probabilă
Semne de afectare a NMC şi NMP în 2 din 6 regiuni (unele semne de NMC fiind rostral situate faţă de cele de NMP)
SLA probabilă, susţinută de testele de laborator
Semne de afectare ale NMC si NMP în o regiune (sau semne de laborator ale afectării NMC într-una sau mai multe regiuni plus proba EMG a denervării acute în 2 sau mai mulţi muşchi situaţi la nivelul a două sau mai multe membre)
SLA posibilă
Semne de afectare ale NMC şi NMP în o regiune

46. ALS EMG criteria for ALS (Lambert):
Normal sensory conduction velocity
Motor conduction velocities normal or nearly normal, depending of the muscke waisting
Fibrilation and fasciculations
Increased amplitude and duration of action potentials
EMG is only accesory to diagnosis in ALS

47. Evaluation and follow up
Global evaluation scales for ALS:
Scoring based on subjective and clinical data
Muscle force measuring
Evaluation of quality of life

48. Treatment Maintaining a good quality of life as long as possible
Prolongation of active life
Prolongation of life expectancy

49. Drug Trials Negative results Other drugs Vitamin E TCH346 Celebrex
Creatine
Indinavir
Topiramate(topamax)
Neurontin
Minocycline
Other drugs
Lithium
Arimoclomol
Ceftriaxone
Manganoporphirin

50. Medical Management Riluzole
The only FDA approved drug for ALS treatment
Approved for use in the US, Europe and Canada –2000
Safe, fairly well tolerated
Moderate efficacy
Expensive ($10,000/year !!)
Prolongs survival by 3-4 months, Cochran review 2005
Side effects include fatigue, nausea, diarrhoea and liver toxicity

51. Muscle weakness Aerobics, exercises to improve flexibility, force
As the disease progresses the rehabilitation programme has to be adapted to possibilities
Establish safe exercises without overexertion
Maintaining independence
Assistive devices (wheel chair, cruches, walker, voice command, lifts, large doors, adapted vehicles)

52. Symptomatic Management
Important part of ALS treatment
Several recent advances for symptom management
Increase in survival and quality of life in patients seen in ALS centers

53. Symptomatic Management
Emotional lability (Pseudobulbar affect)
Spasticity
Cramps and Fasciculations
Difficult to treat generally
-Magnesium –Slow Mag
-Tonic water (quinine)
-Chamomile tea ?
-Low dose baclofen
-Low dose benzodiazepines
Treatment of Pain
Can occur at any stage in the disease
Neuropathic, Musckulosketetal, immobility
Combination of drugs
NSAIDS, Gabapentin, Pregabalin, TCAs
Severe Pain – Narcotics

54. Symptomatic Management
Treatment of Fatigue
Amantidine – dose 100 mg Po twice or three times daily
Modafinil (Provigil)- 100 mg 1-2 times a day
Treatment of other underlying causes
( thyroid dysfunction, anemia etc.)
Insomnia
Bowel and Bladder Dysfunction

55. ALS Management Thick Mucus Production Management of Dysphagia
Increase Hydration
Guiafenesin (robitussin) liquid or pill form
Propananol ?
Suctioning and cough assist device
Management of Dysphagia
Sialorrhea Management
Glycopyrrolate (Robunil)- 1-2 mg every 4 hours
Benztropine, Amitriptyline
Transdermal hyoscine patch
Topical atropine drops - 1% every 4 hours
Botox or Myobloc injection into the salivary glands
Radiation therapy – if very severe
(AAN Practice Parameter- R. G Miller and Colleagues, revised Nov, 2006)

56. Management of Respiratory Failure
FVC checks every 3 months in clinic
BIPAP if FCV < 50 PPV
Invasive ventilation/Tracheostomy when FVC < 30 PPV
Treating underlying infection
Management of patient anxiety
Early signs – sleep problems, morning headache, ortopnea, shallow breath, speech problems, sigh

57. Palliative Care Palliative services involved early
Hospice for patients with end stage ALS
Comfort care and pain management
Early Decision making
Advanced Directives