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Medulla Spinalis, Saraf Perifer, & Otot

dr. Gea Pandhita S, M.Kes, SpS


Bagian Ilmu Penyakit Saraf

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

Medulla Spinalis

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

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

ALS (Amyotrophic Lateral Sclerosis)


Loss of motor neurons in the cortex, brainstem and spinal cord Mix of upper motor neuron and lower motor neuron findings
Weakness, atrophy, fasciculations Slurred speech, difficulty swallowing, shortness of breath

Can start in any extremity or the bulbar musculature Relentlessly progressive

ALS
50 % dead in 3 years, 80% dead in 5 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

Saraf Perifer & Otot

Peripheral Nerve Disorders


Mononeuropathy Mononeuritis multiplex Polyneuropathy (peripheral neuropathy)

Peripheral Nerve Disorders


The spectrum of peripheral nerve disorders includes
Mononeuropathies (entrapment, trauma, etc)
CTS, Bells palsy (?)

Mononeuritis multiplex (DM, vasculitis) Plexopathies (immune, neoplastic) Radiculopathies (discs, immune) Peripheral Neuropathies

Normal peripheral motor nerve anatomy and responses to injury


(Quan, 1999)

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, vasculitis

Polyneuropathy (peripheral neuropathy)


Distal, symmetric

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

Symptoms of a Polyneuropathy
Sensory symptoms
Start in feet, move proximally Hand sxs appear when LE sxs up to knees Positive
Pins and needles Tingling Burning

Negative
Numbness Deadness Like Im walking with thick socks on

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

Guillain Barre Syndrome


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

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

Clinical Manifestation of MG
Sxs worsen with exercise, end of day (Fatigue) Ocular
Droopy eyelids (ptosis) Double vision (diplopia)

Extremity weakness
Arms > legs

Bulbar
Dysarthria Dysphagia

Respiratory
Shortness of breath

Approach to treating MG
Remove any exacerbating factors
Infections, medication, endocrine disease

Acetylcholinesterase inhibitors Plasma exchange/ intravenous immunoglobulin Thymectomy Immunosuppressants


Prednisone Imuran (azathioprin)

Saraf Perifer & Otot

Myopathies

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, when present, are usually late

Classification of Muscle Disease


Dystrophies
Duchennes Muscular Dystrophy Myotonic Dystrophy

Congenital Myopathies
Glycogenoses Mitochondrial

Acquired Myopathies
Polymyositis Dermatomyositis Inclusion body myositis Drug related

Duchennes Muscular Dystrophy


X-linked recessive Absence of dystrophin protein Slow to reach motor milestones, sxs by age 5
All walk, 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

Polymyositis
Presents with proximal muscle weakness in 92% Myalgias in 25% Associated symptoms may include fever, weight loss Slightly increased risk of cancer
Bladder, lung, lymphoma

Biopsy of muscle confirms diagnosis Treatment with immunosuppression


Prednisone Methotrexate

Evaluation of the Patient with Suspected Muscle Disease


Lab
Muscle enzymes (CPK, aldolase) Erythrocyte sedimentation rate (ESR or sed rate) if suspect inflammatory disease Genetic test
Duchennes Myotonic dystrophy

EMG/NCS Muscle biopsy


May provide a definitive diagnosis

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

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