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Grab your Neuro Rapid Review Questions
Cerebral Palsy
- Lifelong physical disability caused by damage to the developing brain; it is non-progressive.
- Typically becomes evident within the first 12 to 18 months of life.
- Etiology: Often idiopathic, but some identified risk factors include:
Risk Factors
- About 50% of CP patients are born premature.
- Low birth weight.
- Maternal infection during early pregnancy.
- Prolonged loss of oxygen during pregnancy or the birthing process.
Clinical Presentation
- Speech and Language Issues
- Dysarthria: Difficulty articulating words.
- Language delay: Inability to speak simple sentences by 24 months.
- Musculoskeletal Symptoms
- Low muscle tone.
- Difficulty holding the head up.
- 75% of patients present with spasticity and contractures, often unilateral.
- Poor muscle coordination.
- Delay in walking development by 12 to 18 months.
- Cognitive and Developmental Delays
- Feeding or Swallowing Difficulties
Labs, Studies, and Physical Exam Findings
- Physical Exam:
- Slow motor development.
- Abnormal muscle tone: either hypertonic or hypotonic.
- Presence of contractures and spasticity.
- Missed developmental milestones.
- Imaging:
- CT or MRI may reveal brain abnormalities or damage.
Treatment
- No definitive cure exists.
- Preventative Measures
- Ensure baby is carried to term.
- Magnesium sulfate administration to prevent preterm labor.
- Medical Treatment
- Benzodiazepines for muscle spasms, e.g., diazepam.
- Botulinum toxin type A injections for spastic muscles.
- Antiepileptic medications to control seizures.
- Surgical Interventions
- Fasciotomy.
- Muscle lengthening procedures.
- Joint replacements.
- Rehabilitative Therapies
- Physical and Occupational Therapy.
- Massage Therapy.
- Educational and Psychosocial Support
- Special education plans.
- Behavioral therapy and counseling.
Differential Diagnosis
- Muscular Dystrophy
- Spina Bifida
- Childhood Stroke
- Other metabolic or genetic disorders
Multiple Sclerosis (MS)
- Demyelinating disease affecting the central nervous system (CNS); exact etiology remains uncertain.
- Autoimmune destruction of myelin.
- Failure of myelinating cells.
- Generally a progressive disease with various clinical courses, most commonly relapsing-remitting.
Risk Factors
- Geographic location: More prevalent in individuals living far from the equator and in Northern European regions. Risk is retained if the person moves after age 15.
- Genetic predisposition.
- Certain viral infections, such as Epstein-Barr virus.
- Smoking.
Clinical Presentation
- Relapsing and Remitting Symptoms: Vary from one area of the body to another during each exacerbation.
- Visual Disturbances: Double vision, nystagmus, partial or total blindness, blurry vision.
- Motor Dysfunction: Focal weakness, gait difficulties, ataxia.
- Sensory Changes: Numbness, tingling.
- General Symptoms: Fatigue, dizziness, vertigo, pain.
- Emotional Changes: Anxiety, depression.
- Other Symptoms: Bladder dysfunction, tremor, headache, seizures.
Labs, Studies, and Physical Exam Findings
- Physical Exam: Neurological assessment.
- Lumbar Puncture: May indicate chronic CNS inflammation, elevated protein levels, and increased leukocytes.
- MRI with Gadolinium: Shows areas of demyelination.
- Evoked Potential Tests: Measures electrical signals in response to stimuli; useful in identifying lesions and nerve damage.
- Blood Tests: To exclude other conditions mimicking MS symptoms.
Treatment Options
- Attack Management
- High-dose corticosteroids: Oral prednisone, IV methylprednisolone (Solu-Medrol).
- Plasmapheresis for severe attacks unresponsive to steroids.
- Disease Modifying Therapies
- Beta interferons.
- Glatiramer acetate (Copaxone).
- Fingolimod (Gilenya).
- Natalizumab (Tysabri).
- Mitoxantrone.
- Teriflunomide (Aubagio).
- Symptom Management
- Physical therapy: Focused on stretching, strengthening, and adaptive devices.
- Dalfampridine (Ampyra): Improves walking speed.
- Baclofen and Tizanidine (Zanaflex): Treat spasticity.
- Amantadine: Addresses fatigue.
- Other medications for mood changes, pain, and bladder/bowel dysfunction.
Differential Diagnosis
- Lupus
- Sjögren’s Syndrome
- Vitamin B12 Deficiency
- Lyme Disease
- Neuromyelitis Optica
Myasthenia Gravis
- Autoimmune neuromuscular disorder affecting the interaction between acetylcholine and its receptors at the neuromuscular junction.
- Acetylcholine usually mediates excitatory signals for muscle contraction.
Clinical Presentation
- Fatigability: Increased muscle weakness that improves with rest.
- Ocular Symptoms: Ptosis (drooping eyelids), diplopia (double vision).
- Oral and Pharyngeal Symptoms: Difficulty in chewing, swallowing, and change in voice quality.
- Respiratory Issues: Difficulty breathing, potentially progressing to respiratory failure (known as myasthenic crisis).
Labs, Studies, and Physical Exam Findings
- Physical and Neurologic Exam
- Often unremarkable but may show easy fatigability.
- Weakness that improves after rest and worsens on repeat exertion.
- Intact reflexes and sensation.
- Blood Tests
- Acetylcholine receptor antibody test.
- Ice Test: Improvement in strength following the application of ice.
- Edrophonium Test: Short-acting anticholinesterase aiding in diagnosis.
- Imaging: CXR, CT, MRI to check for thymoma or other tumors.
- Nerve Conduction Studies: To evaluate nerve-to-muscle signal strength.
- Electromyography (EMG): Shows unresponsive muscle fibers to stimuli.
- Pulmonary Function Tests: To assess respiratory function.
- Muscle Biopsy: Rarely used but may offer additional insights.
Treatment
- Pharmacotherapy
- Acetylcholinesterase Inhibitors: Neostigmine and pyridostigmine prolong the action of acetylcholine.
- Immunosuppressive Agents: Prednisone, cyclosporine, mycophenolate, and azathioprine often used in conjunction with acetylcholinesterase inhibitors.
- Emergent Therapies
- Plasmapheresis and IVIG to remove circulating antibodies.
- Surgical Treatment
- Thymectomy: Particularly in the presence of a thymoma.
Differential Diagnosis
- Lambert-Eaton Myasthenic Syndrome (LEMS)
- Ocular Myositis
- Drug-induced Myopathy
- Guillain-Barré Syndrome
Key Distinctions
- Cerebral Palsy mainly affects motor skills and muscle tone, and it is not progressive.
- Multiple Sclerosis involves an autoimmune attack on the CNS and presents with a wide range of neurological symptoms.
- Myasthenia Gravis focuses on muscle fatigability due to impaired neuromuscular transmission and usually starts with eye symptoms