Spinal chord

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

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

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

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

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

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

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

Tracts of Spinal Cord (Sensory & motor)

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

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

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

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

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

Centromedular formations

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

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

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

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

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

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

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

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

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

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

Syringomyelia

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

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

Friedreich’s disease

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

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

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.

Genetics Autosomal recesive disorder Mutation of frataxin gene (9q13-q21) Long arm of cromosome 9 Prevalence 1 : 50.000 Genetic counselling!

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

Lateral Amiotrophic Sclerosis

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”

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

SLA – clinical picture Muscle weakness Atrophy Stiffness Cramps In 60-85% of pts starts in the limbs and in 15-40% starts with bulbar signs Atrophy Stiffness Cramps Fasciculations; Highly suggestive for ALS if largelly distributed and accompanied by UMN damage siogns

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

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

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

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

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

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

Medical Management Riluzole The only FDA approved drug for ALS treatment Approved for use in the US, Europe- 1996 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

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)

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

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

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

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)

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

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