Chapter 45

Seizure Disorder

• Definition: A seizure is a transient neurological event of paroxysmal nature, characterized by abnormal or excessive action potentials or electrical discharges in the brain.
• Manifestation: Disturbances in skeletal muscle function, sensation, autonomic visceral function, behavior, or consciousness.
• Epilepsy/Seizure Disorder: Term used when there are too many seizure events.
• Causes:
  • Cerebral injury (traumatic lesion, tumor)
  • Metabolic or nutritional disorders (water or ion disturbances, hypoxia, acidosis)
  • Medicine
  • Idiopathic (unknown cause)
• Triggers:
  • Flashing light
  • Fever
  • Low noises
  • Sleep deprivation
  • Hormonal changes (menstruation)
• Physiological Basis:
  • Normally, the cell membrane is impermeable to sodium (Na^+).
  • During depolarization, the membrane becomes permeable, allowing Na^+ to enter the cell.
  • Depolarization: Na^+ entry.
  • Repolarization: Potassium (K^+) exit.
  • Alternation in cell membrane leads to excessive Na^+ entry, causing neurons to become hyperactive and hypersensitive.
  • Epileptogenic focus: A group of abnormal neurons that generate excessive action potentials.
• Aura or Prodrome: A sense of impending seizure (e.g., flashlight, smell, heartbeat).
• Classification:
  • Generalized: The whole brain surface is affected, involving the thalamus and RAS (Reticular Activating System).
  • Partial: Only part of the brain surface is affected.
• Generalized Seizures:
  • Involve thalamus and RAS system, leading to loss of consciousness.
  • Includes absence, atypical absence, myoclonic, atonic, and tonic-clonic seizures.
    • Absence (Petit mal): Staring spells, exclusively in kids, lasting for a few seconds.
    • Atypical Absence: Staring spells with myoclonic jerk.
    • Myoclonic: Sudden, single or several jerks of a muscle group.
    • Atonic: Loss of muscle tonus, causing a drop attack, risky for elderly.
    • Tonic Clonic (Grand mal): Sudden loss of consciousness followed by tonic and clonic phases.
      • Tonic Phase: Muscle rigidity in all extremities, autonomic nervous system involvement (bowel or urinary incontinence); lasts 5-15 seconds.
      • Clonic Phase: Extreme muscle contraction with jerking, tongue biting, eye-rolling, salivation, increased heart rate; lasts up to a couple of minutes.
      • Postictal Phase: Termination of clonic activity; patient regains consciousness (may drift into a coma-like state), confusion, disorientation, amnesia.
• Partial Seizures: Abnormal activity in part of the brain.
  • Simple: No loss of consciousness; may have motor, sensoric, or autonomic symptoms.
  • Complex: Loss of consciousness.
  • Partial Seizure: Starts with activity in one limb and ends with secondary generalization of generalized seizure
• Status Epilepticus: Life-threatening condition with continuous seizures without recovery time; brain doesn't have a relaxation phase, respiratory system in danger.
• Diagnosis:
  • EEG (Electroencephalogram): Special electrical patterns.
  • Laboratory Studies: Metabolic, nutritional, or infection-related causes or toxin in blood
  • LP (Lumbar Puncture): To check for CNS infection.
  • CT or MRI: To identify structural changes.
• Treatment:
  • Maintain open airways during the seizure.
  • Protect patient from injury.
  • Document the course of a seizure (pattern, duration, triggering factors, aura).
  • Anticonvulsant medicine until two years seizure-free, then taper down.
  • Patient education to avoid triggers (stress, alcohol, loud noises).
  • Surgery to remove seizure foci (if medicine isn't working).
  • Nerve stimulation.

Dementia

• A group of diseases associated with pathological disturbances in the brain, characterized by progressive deterioration and declining memory and cognition.
• Delirium: Global mental dysfunction with abrupt onset, worsening at night, disturbed consciousness, hallucination, muscle activity.
• Types of Dementia:
  • Alzheimer's disease (most common, ~60-65%).
  • Vascular dementia.
• Alzheimer's Disease:
  • Neuron degeneration in temporal and frontal lobes.
  • Hallmarks: Brain atrophy, amyloid plaques, and neurofibrillary tangles.
    • Brain Atrophy: Neuron death, flat gyra, wide sulcus, wide ventricles filled with CSF.
    • Amyloid Plaques:
      • APP (Amyloid Precursor Protein) in the cell membrane.
      • Beta Amyloid: Fragments of APP that are not cut correctly by enzymes that, along with other inflammatory cells, making amyloid plaques.
    • Neurofibrillary Tangles:
      • Hyperphosphorylated tau protein, tangled together and become neurofibrillary tangles in axon heloc.
      • Impulse generation in axon hillock goes down.
  • Low production of acetylcholine.
  • Triggering factors: Inflammatory reactions, genetic factors, environmental triggers, aging.
  • Starts with short-term memory loss, progressing to long-term memory loss.
  • The patient becomes unable for self-care.
  • Must exclude depression and psychosis before diagnosing Alzheimer's.
• Vascular Dementia:
  • Cerebrovascular insults (atherosclerosis, hypertension, diabetes) decrease blood supply to the brain.
  • Risk factors: Stroke, hypertension, diabetes.
  • Early manifestation is memory loss & decline in abstract thinking.
  • Declining ability to function at work and home.
  • Anxiety when changing environment.
  • Need assistance for ADLs (Activities of daily living).
  • Weight loss.
  • Loss of control of bladder and bowel movements.
  • Personality or behavior changes.
• Diagnosis:
  • Exclude delirium.
  • Complete family history and physical examination.
  • CBC and chemistry panel.
  • Exclude conditions like hypothyroidism, B12 deficiency, or syphilis.
  • Chest X-ray and CT.
  • LP for suspected CNS infection.
  • MMSE (Mini-Mental Status Exam): 32 questions checking abstract and spacial thinking.
• Management:
  • Early diagnosis and intervention.
  • Medication: Acetylcholinesterase inhibitors (Tacrine, Donepezil, Rivastigmine, Galantamine for mild to moderate Alzheimer's) slow down acetylcholine removal and mementine, blocking glutamate receptors (NAMBDA receptor antagonist) for severe Alzheimer's.
  • Care for family and caregivers.

Parkinson's Disease

• Etiology: Idiopathic (vast majority), acquired, or drug-induced.
• Cause: Dopamine deficiency due to destruction of substantia nigra in basal ganglia.
• Characteristics: Impairment of movement, difficulty initiating and controlling movements.
• TRAP:
  • Tremor (rest tremor).
  • Rigidity (cogwheel rigidity).
  • Akinesia (bradykinesia).
  • Posture (unstable).
• Symptoms are based on low activity on dopamine pathway
• Facial mask, shuffling gait, and no arm swing during clinical presentation
• Treatment:
  • Dopamine-like medicine.
  • Dopamine agonists.
  • MAO (monoamine oxidase) inhibitors.
  • Anticholinergics.
  • Antidepressants.
  • Ablative surgery or deep brain stimulation.

Cerebral Palsy

• Definition: Group of crippling syndromes appearing in kids, permanent damage to upper motor neurons.
• Classification: Spastic, ataxia, and dyskinesia.
• Etiology: Could be prenatal infections or disease of the mother, some trauma during delivery,
• Treatment: Muscle relaxants, anticonvulsants, orthopedic surgery, casts and braces, Botox.

Hydrocephalus

• Abnormal accumulation of CSF (Cerebrospinal Fluid) in the CNS.
• Types:
  • Normal Pressure Hydrocephalus: Increased CSF volume without increased intracranial pressure (gait instability, urinary incontinence, dementia).
  • Obstructive Hydrocephalus: Blockage in CSF circulation, increases intracranial pressure.
  • Non-Obstructive (Communicating) Hydrocephalus: Abnormal CSF absorption.
• Treatment for obstructive hydrocephalus is a surgery to remove obstruction.
• Shunts: Tube from lateral ventricle to abdomen to drain CSF.

Cerebellum Disorders

• Cerebellum functions: Balance, posture reflexes, muscle tonicity, and coordination.
• Cerebellum disorders will cause muscle coordination and tonicity problem.
• Etiology: Abscess, hemorrhage, tumor, trauma, or viral infection.
• Symptoms: Ataxia, hypotonia, intention tremors.
• Treatment depends on the cause.

Spinal Cord and Peripheral Nerve Disorders

• Multiple Sclerosis (MS): Demyelination of axons in the CNS, causes inflammation and scarring.
• MS will affect optic and oculomotor nerves and the spinal nerve tracts
• Exacerbation and remissions, affects young adults, autoimmune disorder.
• Lost in space and time symptom.
• Symptoms: Blurred vision, muscle coordination problems, sensory differences, bowel or bladder control (latter).
• Diagnosis: MRI can show plaque in myoeline sheaths
• Treatment: No cure; steroids for suppressing the immune system during exacerbations, immune-modifying drugs.
• Spina Bifida: Developmental anomaly where vertebra lamina doesn't close properly, spinal cord or meninges can protrude out.
  • Occulta (not visible), cystica (meningocele, meningomyelocele).
  • Lack of folic acid causes some spina bifida
  • Diagnosis: Ultrasound, alpha-fetoprotein.
  • We have the option of surgery and c section.
  • Advise patients to have supplement with folic acid.
• Amyotrophic Lateral Sclerosis (ALS): Damaging both upper and lower motor neurons.
• Lateral: inflammation makes lateral tracts hard
• Muscle is getting weak and atrophy due to muscle atrophy.
• Weakness and wasting of upper extremities, impaired speech, problem with swallowing, respiratory problems.
• Clinical mass manifestation: atrophy of muscle and weakness of muscles
• Hyperreflexia in weak muscles needs to be watched closely, suspecting ALS.
• 5 years prognosis is very poor after diagnosis.
• Treatment: Riluzole (glutamate inhibitor).
• Spinal Cord Injury:
  • Etiology: Traumatic accidents, fall or penetrating wounds.
  • Spinal cord injury can happen for overflexion, hyperextension, and compression
  • Inflammation will cause the swelling which also put pressure around the spinal cord itself.
  • Primary injuries: hyperflexion, hyperextension, and compression.
  • Secondary injury: Ischemia and inflammation.
    • 
      Immediate after spinal cord injury. you will have temporary loss of reflexes and neurological function after that injuries
• This is spinal shock with muscle becomes very flaccid. And all autonomic reflexes will disappear
  It including bladder end intestinal re fluxes
  When the reflexes are returning, and this placid becomes muscle which were very relaxed
• Now it becomes very contracted. So that's the mark of this end of Spinal Shock
• Neurogenic shock: Loss of sympathetic nervous system, distributive shock.
  • Peripheral vasodilation causes hypertension, bradycardia; life-threatening.
• Autonomic Dysreflexia: Complication post-spinal shock where the injury at T6 or above.
  • A reflex to sympathetic stimulation
    Can cause hypertension, headache, bradycardia, and flushing above T6 and then you have vasoconstriction, sweating, and piloerection, which is goosebumps will be all these below T6
• Treatment: Stabilize spinal vertebrae through external or internal fixation, high-dose prednisolone for secondary injury prevention, intensive rehabilitation.
• Guillain Barre: Demyelination in peripheral nervous system.
  Guillain barre is also an auto gnomic nervous system damage.
• It could be either B T or B cell or both of them which are mediating the this attack.
• ascending paralyze It Start from your feet and going up towards your head
  It affect your diaphragm, then, you know, your rest, ventilation and respiratory function is in danger.
• Ascending paralysis from lower extremity to spinal area and ascending to higher muscles
• The old name for that is the polyneuropathy or Polyradicular neuropathy.
  Most of the cases are idiopathic
• The self limiting and recovery will occur spontaneously.
  If At some point if, it's effect your diaphragm, then, you know, your rest, ventilation and respiratory function is in danger.
  so we are monitoring patients very very closely.

As mentioned, lower extremity, and it goes out to proximal spinal

It can give weakness or paralysis and especially we need to keep an eye on respiratory muscles

• Campullbacter gastrointestinal problems are linked to Guillain Barre.
• Diagnosis: Patient story physical examination and nerve conduction studies.
• Plasma freezing, do is remove the bodies or we are giving Ivy 82 patients
• Treatment: Supportive care, plasmapheresis, or IVIg (immunoglobulin).
• Bell's Palsy: Neuropathy of facial nerve causing one-sided facial paralysis.
  facial nerve is going. This is, let me change the color here. Here's a ear canal and facial nerve is running through
  Sometimes there is a pressure here for some reason in that canal.
• So we can do MRI to see if there is something which is blocking or comprising this nerve.
• Most connected to virus infection It can develop up to two days
• Symptoms: Unilateral facial weakness, facial droop, reduced eye blink, hyperacusis.
• Clinical signature with patient history and physical finding., We, can, put in the diagnosis
• Diagnosis: Patient history, physical exam, MRI to check for nerve compression.
• Treatment: Self-limiting and providing support. In Some physicians also give antiviral medication like Asycalvia