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Medulla Spinalis, Saraf Perifer, & Otot

dr. Gea Pandhita S, M.Kes, SpS
Bagian Ilmu Penyakit Saraf Fakultas Kedokteran – Universitas YARSI

Medulla Spinalis

Medulla Spinalis

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Neoplasma : Primer & Metastasis Lokasi .Absces Vaskular = Tumor = .Medulla Spinalis Infeksi = .HNP .Spondilosis Trauma = .Non-neoplasma : Degeneratif = .Mielitis .Spondilitis .

Saraf Perifer & Otot .

Disorders of the Motor Unit
• • • • Motor neuron disease Peripheral nerve disorders Neuromuscular junction disease Muscle disease

Saraf Perifer & Otot

Motor Neuron Disease
• Diseases that can involve Betz cells of the motor cortex, the lower CN motor nuclei, the CST, and/or the anterior horn cells
– Amyotrophic Lateral Sclerosis (ALS) – Progressive bulbar palsy – Progressive muscular atrophy, spinal muscular atrophy – Primary lateral sclerosis

shortness of breath • Can start in any extremity or the bulbar musculature • Relentlessly progressive . atrophy. fasciculations – Slurred speech.ALS (Amyotrophic Lateral Sclerosis) • Loss of motor neurons in the cortex. difficulty swallowing. brainstem and spinal cord • Mix of upper motor neuron and lower motor neuron findings – Weakness.

ALS • 50 % dead in 3 years. 5-10% live more than 10 years • Death usually from respiratory failure • Etiology still only theoretical – – – – Excess glutamate Oxidative stress Free radicals Mitochondrial dysfunction . 80% dead in 5 years.

Saraf Perifer & Otot .

Peripheral Nerve Disorders • Mononeuropathy • Mononeuritis multiplex • Polyneuropathy (peripheral neuropathy) .

neoplastic) Radiculopathies (discs.Peripheral Nerve Disorders The spectrum of peripheral nerve disorders includes – Mononeuropathies (entrapment. Bell’s palsy (?) – – – – Mononeuritis multiplex (DM. immune) Peripheral Neuropathies . vasculitis) Plexopathies (immune. etc) • CTS. trauma.

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Normal peripheral motor nerve anatomy and responses to injury (Quan. 1999) .

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symmetric . vasculitis • Polyneuropathy (peripheral neuropathy) – Distal.Peripheral Nerve Disorders • Mononeuropathy – Pattern of weakness and sensory loss conforms to the distribution of a single nerve • Carpal tunnel syndrome • Peroneal palsy at the fibular head • Mononeuritis multiplex – Multiple nerves affected in a random pattern • Acute onset. frequently painful • Diabetes mellitus.

Polyneuropathies • Can affect different types of fibers – Autonomic – Motor – Sensory • Large well myelinated • Small poorly myelinated or unmyelinated .

move proximally – Hand sxs appear when LE sxs up to knees – Positive • Pins and needles • Tingling • Burning – Negative • Numbness • Deadness • “Like I’m walking with thick socks on” .Symptoms of a Polyneuropathy • Sensory symptoms – Start in feet.

Polyneuropathy Symptoms • Motor – Weakness first in feet • Tripping • Turn ankles – Progress to weakness in hands • Trouble opening jars • Trouble turning key in lock .

Polyneuropathy: Signs • Distal sensory loss – Large fiber – Small fiber • Distal weakness and atrophy • Decreased or absent reflexes – Ankle jerks lost first .

Classification of Polyneuropathies • By types of fibers involved – – – – Pure sensory Sensory motor Pure motor Autonomic • By pathology – Demyelinating – Axonal – Mixed • By tempo – Acute – Subacute – Chronic .

Acute Polyneuropathies • Guillain Barre Syndrome • Porphyria – Neuropathy. psychiatric disorder. unexplained GI complaints • Toxins – Glue sniffing (n-hexane) – Arsenic .

Guillain Barre Syndrome • Definition of GBS – acute demyelinating disorder. primarily involving the peripheral nervous system (PNS) – degree and location of disability depends on the myelinated nerves involved: MOTOR .

The condition is often designated as the Landry-Guillain-Barré-Strohl syndrome in recognition of the descriptions provided by these authors .Guillain Barre Syndrome an inflammatory disease of the peripheral nervous system characterized by lymphocytic and macrophagic infiltration with destruction of myelin.

Guillain Barre Syndrome .

Guillain Barre Syndrome • Most common cause of rapidly progressive weakness • Demyelinating neuropathy • Ascending weakness which may include cranial neuropathies • Exam reveals symmetric weakness with areflexia and large fiber sensory loss • Bowel and bladder usually preserved .

Guillain Barre Syndrome • Respiratory failure can be precipitous • Other causes of morbidity and mortality – Autonomic instability – DVT – Infection • Immune mediated. may be post infectious • Treatment – Plasma exchange – Intravenous immunoglobulin .

Guillain Barre Syndrome .

Subacute Polyneuropathies • Vasculitis – Can be isolated to peripheral nerves or part of a more systemic process • Paraneoplastic – May be presenting symptom of the cancer • Chronic inflammatory demyelinating polyneuropathy – With or without a gammopathy • Toxins • Drug .

Chronic Polyneuropathies • Metabolic – – – – Diabetes mellitus Chronic renal failure Chronic liver failure Thyroid disease • Nutritional – B12 deficiency • Infections – HIV – Leprosy • Inherited .

Evaluation of a Polyneuropathy • Lab work • Nerve conduction study/electromyography – Distinguishes between axonal and demyelinating – Helps ascertain severity • Nerve biopsy – Frequently non-diagnostic – Can establish the dx in certain disorders. such as vasculitis and amyloidosis .

Saraf Perifer & Otot .

Disorders of the Neuromuscular Junction .

NMJ • Pre-synaptic – Lambert Eaton myasthenic syndrome – Botulism • Post-synaptic – Myasthenia Gravis .

Myasthenia Gravis • Antibody that alters the acetylcholine receptor – Binding – Blocking – Modulating • Antibody detected in – 50% of pts with pure ocular MG – 90-95% of pts with generalized MG .

end of day (Fatigue) • Ocular – Droopy eyelids (ptosis) – Double vision (diplopia) • Extremity weakness – Arms > legs • Bulbar – Dysarthria – Dysphagia • Respiratory – Shortness of breath .Clinical Manifestation of MG • Sxs worsen with exercise.

endocrine disease • Acetylcholinesterase inhibitors • Plasma exchange/ intravenous immunoglobulin • Thymectomy • Immunosuppressants – Prednisone – Imuran (azathioprin) .Approach to treating MG • Remove any exacerbating factors – Infections. medication.

Saraf Perifer & Otot .

Myopathies .

when present.Clinical Manifestations of Myopathies • Proximal muscle weakness – Waddling gait – Difficulty climbing stairs – Trouble lifting arms over head • Cramps with the metabolic myopathies • Myalgias with the inflammatory myopathies • Swallowing and breathing difficulties. are usually late .

Classification of Muscle Disease • Dystrophies – Duchenne’s Muscular Dystrophy – Myotonic Dystrophy • Congenital Myopathies – Glycogenoses – Mitochondrial • Acquired Myopathies – – – – Polymyositis Dermatomyositis Inclusion body myositis Drug related .

may never run – End up in wheelchair by age 10-12 • Steroids may delay time until wheelchair bound • Muscles replaced by fat may appear hypertrophic • Frequently mildly mentally retarded • Life expectancy < 20 years with death related to respiratory failure or cardiomyopathy . sxs by age 5 – All walk.Duchenne’s Muscular Dystrophy • X-linked recessive • Absence of dystrophin protein • Slow to reach motor milestones.

Polymyositis • Presents with proximal muscle weakness in 92% • Myalgias in 25% • Associated symptoms may include fever. lung. weight loss • Slightly increased risk of cancer – Bladder. lymphoma • Biopsy of muscle confirms diagnosis • Treatment with immunosuppression – Prednisone – Methotrexate .

aldolase) – Erythrocyte sedimentation rate (ESR or sed rate) if suspect inflammatory disease – Genetic test • Duchenne’s • Myotonic dystrophy • EMG/NCS • Muscle biopsy • May provide a definitive diagnosis .Evaluation of the Patient with Suspected Muscle Disease • Lab – Muscle enzymes (CPK.

Extremity Weakness ALS Polyneuropathy LEMS Random Distal> Proximal LE > UE Prox>distal UE>LE +/-prox>distal Prox>distal CN Reflexes Sensation yes rare Increased Decreased distally Decreased or absent Normal or dec Normal Lost distally > proximally Normal rare MG yes Normal Myopathy occ Normal or dec Normal .

Terima Kasih .