Neuro 1😍

Mesial temporal lobe epilepsy

• Lesion at Medial temporal lobe → No explicit memory
• Complex partial seizure.
• Uncinate fits (Smell / taste hallucination).
• Deja vu (Unfamiliar environment seems familiar).
• Jamais vu (Familiar environment seems unfamiliar).

Carbamazepine

• Causes leucopenia, aplastic anemia, hepatotoxicity as a side effect.

• Patient of liver damage
• oxcarbazepine > carbamazepine

Levetiracetam

• Best safety profile

• Preferred in elderly individuals and pregnancy

Lamotrigine

• DOC: For focal seizures in elderly (along with levetiracetam).

• Side effects:
• Steven Johnson Syndrome
• Toxic Epidermal Necrolysis
• These side effects can be avoided by gradually increasing the dose

• Slowly Leave (Leviteracetum) Lame (Lamotrigine) Elderly with lame skin (SJS, TEN)

Absence Seizures / Petit Mal Epilepsy

• Characterized by vacant staring spells followed by blinking episodes.

• No tonus or clonus will be present.

• Seen in the pediatric age group.

• No tonic or clonic phase.

• Vacant staring spells are seen in children
• (The child is called a daydreamer/absent-minded by parents).

• Exacerbated by Hyperventilation

• Blinking of eyes seen during the episode.

• Seizure episodes can occur several times a day.

• No post-ictal deficit is seen.

• The child is unresponsive during the episode, but the postural tone is maintained.

• Absence seizures can be triggered by hyperventilation or bright light (photic stimulation).

• EEG shows a 3/sec spike and slow wave pattern.

Treatment

• Valproate.

• Ethosuximide is used in children less than 5 years old as valproate may cause hepatotoxicity.

Important Information on Seizure Types

• Most common type of seizures in neonates: Subtle Seizures.

• Most common type of seizures in a child: Febrile Seizures.

• Most common type of epilepsy in a child: Benign Rolandic Epilepsy.

• Most common type of epilepsy in adults: GTCS.

Subtle Seizure

• Most common type of seizure in neonates.

• There will be no convulsions, only jitteriness is seen like tremors of lips and fingers.

• Convulsions are not seen in neonates as CNS is not fully developed in neonates.

Benign Rolandic Epilepsy

• It is the most common type of epilepsy seen in children.

• It is a focal seizure.

• An uncontrollable twitching on one side of the face and drooling of saliva occurs.

• Mother may give a similar history of saliva staining of pillow & may fail to notice episodes that occur during sleep.

Clinical Features

• 30-year-old man presented with complaint of myoclonic jerks + dementia.

• Myoclonic jerks may be produced in response to loud sound:
• Startle myoclonus.

• History suggests consumption of poor-quality beef
• eating tainted beef/ infected beef with prions.

• Transmissible Spongiform Encephalopathy (TSE).

• Bovine spongiform encephalopathy
• Spongiform vacuolations brain
• Prion → Praani () infected a Kuru () → made it spongy (spongy vacuolations) like → we took it to congo (congo red) and Passed (PAS) it to them

Pathology

• Prion disease (prion particle - smallest infectious particle).

• Prion: Protein coat only, no DNA/RNA.

• Resistant to 121°C heat.

• Kill by 134°C for 1.5 hrs (Autoclaving)

• Source: Tainted beef

• Has a long incubation period

• Prion particles → enter the GIT → enter blood stream → cross the blood brain barrier.

• PrPC → PrPSC is the abnormal folded protein
• accumulates as β Pleated structures

• start accumulating in the cytoplasm of neurons
1. Cause neurodegeneration
• dementia
2. Abnormal firing of neurons
• results in myoclonic jerks

Manifestations:

• Myoclonic jerks
• startle myoclonus

• Dementia.

• Gait ataxia.

• Incapacitation / Bedridden.

• Death due to pneumonia in Parkinsonism, Alzheimer's, VCJD.

MRI head:

• Increased intensity of basal ganglia

• Cortical Ribboning

• Hockey stick sign → Pulvinar Nucleus of thalamus

• Hockey stick sign seen in:
• In MRI: VCJD
• In ECG: Digoxin.
• In Echo: Mitral stenosis.
• In OBG: USG Abdominal circumference

CSF Examination

• Shows protein 14.3.3

EEG Finding

• Periodic sharp wave complexes.
• Keyword in MCQ, to identify VCJD
• Mnemonic:
• Variant = Periodic
• Crude = Sharp

Important Information

• Periodic lateralized epileptiform discharge (PLED)
• is seen in HSV encephalitis.
• Mnemonic: His wife → Period late → she seizures

Prion Diseases

• Sreelankan (Gerstman Schlankler Syndrome) cannibals (Kuru ds) sleep (familial fatal insomnia)

• Variant Creutzfeldt-jakob disease.

• Classical Creutzfeldt-jakob disease.
• From Human GH Inj → Prev taken from cadaveric pituitary
• Human to human transmission
• Not todays world

• KURU Disease
• (Cannibals).
• Specifically shows Kuru's plaque.
• Positive for Congo red and PAS

• Familial fatal insomnia.

• Gerstman Schlankler Syndrome.
• Disease 4 & 5 are genetic (Chromosome 20 defect).

Revise Epilepsy

SSLC / SCENE Genes

West Syndrome/ Infantile Spasm/ Salaam Seizures

• It is seen in infants (<1 years age).

• Triad
1. Infantile spasms (Salaam Attacks/flexor spasms).
2. Global developmental delay.
3. Hypsarrhythmia (on EEG).

EEG Findings

• Hypsarrhythmia: Chaotic pattern is seen.

• High amplitude polyspikes are seen.

• Frequency is low (delta grade).

• CRH (Corticotropic Releasing hormone) is involved

Management

• Inj. ACTH (it downgrades the CRH).
• INICET → Steroids

• VIGABATRIN: Tuberous sclerosis.

Important Information:

• Various drugs of choice in pediatric neurological conditions:
• Absence seizures: Valproate/Ethosuximide
• Juvenile myoclonic epilepsy: Valproate
• West syndrome: Inj. ACTH
• Infantile spasms in Tuberous sclerosis: Vigabatrine

SSPE (Sub Acute Sclerosing Panencephalitis)

• Rare, fatal neurodegenerative complication of measles

• Appears 8 to 10 years after initial infection

• Virus crosses blood brain barrier and causes neurodegeneration.

CF:

• 8 years old unimmunized Previously child was completely normal
• Now presents with not able to understand things taught in school.
• Scholastic performance of child keeps on decreasing

• EEG shows burst suppression
• periods of high-voltage electrical activity
• alternating with periods of low-voltage activity
• Also seen in HIE stage 3

Investigation

• Diagnosis:
• CSF and Blood anti-measles antibody is done.

Neurological progression:

• Myoclonic jerks

• Sudden falls

• Choreoathetosis

• Dystonia

• Rigidity

• Course: Progressive downhill

Treatment

• No effective treatment currently available

JANZ Syndrome/ Juvenile Myoclonic Epilepsy

• Genes Associated
• GABRA-1 (GABA receptor),
• CACNB (calcium receptor genes)
• CASR (cal sensing receptor)
• Mnemonic: Janzi → Gabri (GABRA 1) kk Cash (CASR) illathapo → Seizure vann

• Seen in adolescents of age group 10-19 years of age.

• Parents give history of recurrent slipping of things from hands of child.
• Epilepsy (myoclonic)

• Early morning hours

• Due to sleep deprivation

• Later myoclonic + GTCS/absence seizures

• Good prognosis

EEG Finding

• 4-6 polyspike pattern.

• More chances of developing GTCS in later years of life.

Treatment

• Sodium valproate

4 types of Arnold Chiari malformations

Cerebellar tonsil herniation

Syringomyelia:

• Causes central canal lesion

• fluid-filled cavity (syrinx) within the spinal cord.

• A/w Chiari malformation Type 1, trauma, or tumors

• damage to spinothalamic tract fibers crossing the anterior commissure

Central canal lesion

• B/L loss of pain & temperature initially
• due to disruption of decussating fibers
• Preserved touch and vibration (Proprioception)

• Cape-like distribution Sensory loss over the nape of the neck

• First affect Cervical spinothalamic

• f/b LMN dysfunction
• due to anterior horn cell compression if large lesion
• features
• muscle wasting,
• weakness
• hyporeflexia in the upper extremities.

• Autonomic dysfunction:
• Bowel and bladder dysfunction in advanced cases

• UL > LL

Anterior canal lesion

• Pain and temperature loss + Motor loss together appears

• Involves anterior 2/3rd of column

• UL = LL

• Preserved touch and vibration (Proprioception)

Myelography

• Contrast given in subarachnoid space.

• Appears as white contrast surrounding spinal cord.

• Contrast can also appear black.

• CT Myelogram
• Shows white contrast.

• MR Myelogram
• Does not show white bone.
• Is a T2 weighted MRI.
• Useful to see CSF.

STIR

• T2 (-) Fat

• For bone marrow edema

• Uncal herniation:
• Herniation of uncus (medial temporal lobe)

• Progression:
• Affect Parasympathetic fibers of ipsilateral cranial nerve III
• Called "ipsilateral blown pupil" or "false localizing sign"
• C/L corticospinal tract/crus cerebri compressed
• I/L UMN palsy

• Kernohan’s notch phenomenon:
• False localizing sign
• Hemiparesis appears ipsilateral to lesion
• instead of expected contralateral

Key signs:

• Ipsilateral pupil dilatation → Hutchinson pupil

• Ipsilateral UMN palsy

Hutchinson's

• H → Herpes Zoster Ophthalmicus

• U → subUngual Melanoma (superficial spreading melanoma)
• Hutchinson sign

• T → Triad → congenital syphillis
• Peg shaped teeth
• Interstitial Keratitis (IK + SNHL)
• SNHL

• CH → Chauffeur's Fracture/Backfire Fracture
• Intra articular #

• Son → looking older → Hutchison Gilford
• LMN A gene defect (laminopathy).
• Progeria (onset: Child)

• PUPIL → Hutchinson Pupil
• Herniation of uncus (medial temporal lobe) → compresses ipsilateral CN III → same side pupillary dilatation.
• Kernohan’s notch phenomenon:
• False localizing sign
• Ipsilateral pupil dilatation
• Ipsilateral UMN palsy