Tag: Neurology

Spinal cord

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Check Trauma (Spinal cord injuries)
This section will be updated.
SC compression
?
·       Spinal injury (MVA)
·       Malignancy (Lung, breast, prostate cancers; myeloma)
·       Infection (eg, epidural abscess)
S/S
·       Gradually worsening,
·       Severe local back pain
·       Pain worse in the recumbent position/at night
·       Early signs: Symmetric lower extremity weakness,  ⤵️DTR
·       Late signs: Bilateral Babinski reflex, ⤵️ rectal sphincter tone, paraparesis/paraplegia
·       ⤴️ DTR, sensory loss
Tx
·       Emergency MRI 🚨
·       IV GCS

 Spinal epidural abscess
#
• Staphylococcus aureus (65%)
• Inoculating sources
    o Distant infection (eg, cellulitis, joint/bone)
    o Spinal procedure (eg, epidural catheter)
    o Injection drug use
Clx
Classic triad
·       Fever (—50%)
·       Focal/severe back pain
·       Neurologic findings (eg, motor/sensory change,
·       bowel/bladder dysfunction, paralysis)
Dx
• ⤴️ESR
• MRI of the spine
Tx
Broad-spectrum antibiotics (eg. vancomycin plus ceftriaxone)
• Aspiration/surgical decompression
Cauda equina syndrome
Conus medullaris syndrome
Usually bilateral, severe radicular pain
Sudden-onset severe back pain
Saddle hypo/anesthesia
Perianal hypo/anesthesia
Asymmetric motor weakness
Symmetric motor weakness
Hyporeflexia/areflexia
Hyperreflexia
Late-onset bowel & bladder dysfunction
Early-onset bowel & bladder dysfunction

Dementia

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 Cognitive impairment in elderly patients
Normal aging
·       Slight decrease in fluid intelligence (ability to process new information quickly)
·       Normal functioning in all activities of daily living
Mild neurocognitive disorder
(mild cognitive impairment)
·       Mild decline in 21 cognitive domains
·       Cognitive testing <26
·       Normal functioning in all activities of daily living with compensation
Major neurocognitive disorder
(dementia)
·       Significant decline in 21 cognitive domains
·       Irreversible global cognitive impairment
·       Marked functional impairment
·       Chronic & progressive, months to years
MDD
·       Reversible mild-moderate cognitive impairment
·       Features of depression (mood, interest, energy)
·       Episodic, weeks to months
Dementia
AD
·       Early, insidious short-term memory loss
·       Language deficits & spatial disorientation
·       LATE personality changes
Vascular
·       Stepwise decline
·       Early executive dysfunction
·       Cerebral infarction &/or deep white matter
·       changes on neuroimaging
FT
·       Early personality changes
·       Apathy, disinhibition & compulsive behavior
·       FT atrophy on neuroimaging
Lewy
·       Visual hallucinations
·       Parkinsonism
NPH
·       Ataxia early
·       Urinary incontinence
·       Dilated ventricles
CJD
·       Behavioral changes
·       Rapid progression
·       Myoclonus
Differential diagnosis of dementia subtypes
Alzheimer disease
• Early, insidious short-term memory loss
• Language deficits & spatial disorientation
• Later personality changes
Vascular dementia
• Stepwise decline
• Early executive dysfunction
• Cerebral infarction &/or deep white matter changes on neuroimaging
Frontotemporal dementia
• Early personality changes
• Apathy, disinhibition & compulsive behavior
• Frontotemporal atrophy on neuroimaging
Lewy body dementia
• Visual hallucinations
• Spontaneous parkinsonism
• Fluctuating cognition
Normal pressure hydrocephalus
• Ataxia early in disease
• Urinary incontinence
• Dilated ventricles on neuroimaging
Prion disease
• Behavioral changes
• Rapidly progressive
• Myoclonus &/or seizures
Normal pressure hydrocephalus
Clx 3W
·       Gait instability (wide-based) with frequent falls
·       Cognitive dysfunction
·       Urinary urgency/incontinence
·       Depressed affect (frontal lobe compression)
·       IJMN signs in LL✳️
Dx
·       Miller Fisher test: improvement in gait with spinal fluid removal
·       MRI: Enlarged ventricles out of proportion to the underlying brain
·       atrophy
Tx
·       Ventriculoperitoneal shunting
Creutzfeldt-Jakob Disease
Clx
·       RAPID DEMENTIA
·       AND
·       ≤2 out of 4 clinical features:
·       Myoclonus
·       Akinetic mutism
·       Cerebellar or visual disturbance
·       Pyramidal/extrapyramidal dysfunction (eg, hypokinesia)
🏥
·       ≤1 of the following:
·       Periodic sharp wave complexes on EEG
·       ⊕14-3-3
·       Caudate nucleus/putamen MRI findings
1.     Neuropathology: spongiform changes, neuronal loss without inflammation
HIV Neurocognitive disorders
S/S
·       Impaired memory & attention/concentration
·       Personality & behavior changes
·       Motor symptoms (Ataxia, slowed movement)
Severity
·       Asx neurocognitive impairment
·       Mild neurocognitive disorder
·       HIV-associated dementia
RF
·       Long-standing HIV disease
·       Age 250
·       CD4 count <200
Dx
Clinical presentation
MRI: DIFFUSE IN WHITE MATTER (vs PML: local)
Neuropsychological testing
HIV-associated dementia (HAD)
·       CD4+ cell count of <200/mm3
·       Onset is typically subacute and characterized by increasing apathy and impaired attention.
·       Subcortical (eg, basal ganglia, nigrostriatal) dysfunction
·       occurs early with symptoms such as slowed movement and difficulty with smooth limb movement.
·       This is followed by significant cortical neuronal loss and memory decline.

Gait Disorders

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 Gait Disorders 
 Features
Sx
Causes
Cerebellar
Ataxic: Staggering, wide-based
Dysdiadochokinesia, dysmetria,
NYSTAGMUS, Romberg sign
·       Cerebellar degeneration
·       Stroke
·       Alcohol
·       B12 Def
Gait apraxia
(frontal gait)
Magnetic (freezing): Start & turn hesitation
Dementia, incontinence, frontal lobe signs
·       Normal-pressure hydrocephalus
·       Degeneration
Parkinson disease
Short steps, shuffling
Bradykinesia, resting tremor, postural
instability, decreased arm swing
·       Degeneration of dopaminergic neurons
Steppage
Footdrop, excessive hip & knee flexion while walking, slapping quality, falls
Distal sensory loss & weakness
Nerve injury
Vestibular
Unsteady, falling to one side
VERTIGO
·       Acute labyrinthitis
·       Méniére disease

Alcoholic cerebellar degeneration
#
·       >10 years heavy alcohol use
·       Degeneration of Purkinje cells (cerebellar VERMIS)
S/S
·       Weeks to months
·       Wide-based gait
·       Incoordination in legs
·       Ⓝ Cognition
Dx
Clx:
·       Impaired tandem walking
·       Heel-knee-shin
·       Preserved finger-nose testing
CT/MRI – cerebellar atrophy
Tx
·       Alcohol cessation.
·       Nutritional supplements
·       Ambulatory assistance devices (eg, walker)

Neurocutaneous Syndromes

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 Neurofibromatosis types 1&2  
Dx
NF1 (von Recklinghausen disease) NF2 (central neurofibromatosis)
NF2(central neurofibromatosis)
Gene
NF1 tumor suppressor gene;
– Protein: neurofibromin
NF2 tumor suppressor gene;
-Protein merlin
#
Chromosome 17
Chromosome 22
Clx
1.     Café-au-lait spots
2.     Multiple neurofibromas
3.     Lisch nodules
• Bilateral acoustic neuromas

 Sturge-Weber Syndrome
Path
Mutation in GNAQ gene
S/S
·       Port wine stain (trigeminal N /CN VI/V2)
·       Leptomeningeal capillary-venous malformation
·       SZ ± hemiparesis
·       Intellectual disability
·       Visual field defects
·       Glaucoma
Dx
 MRI with contrast
Tx
·       Laser therapy
·       Antiepileptic drugs
·       Intraocular pressure reduction
 Tuberous Sclerosis
?
·       Neurocutaneous autosomal dominant 
·       hamartin and tuberin form a complex that down regulates mTOR signaling
·       when this pathway is 🆎 → hamartomas everywhere
S/S
·        infantile spasms in infants and have hypsarrhythmias on EEG
·       SZ
·       Arrhythmia 2ndary to cardiac rhabdomyomas
Dx
Genetic testing
Tx
Corticotropin for infantile spasms

Seizures

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Absence SZ
Clx
• Sudden impairment of consciousness (“staring spells”)
• Preserved muscle tone
• Unresponsive to tactile/verbal stimulation
• Short duration (<20 seconds)
• Simple automatisms frequently present
• Easily provoked by hyperventilation
Dx
EEG: 3-Hz spike-wave discharges during episodes
Co-dz
• ADHD
• Anxiety
Tx
Ethosuximide

Focal SZ
?
• Originates from 1 cerebral hemisphere
• +/- Loss of consciousness
Types
• Motor: Jacksonian march, turning of eyes/head/trunk
• Sensory: Paresthesias, vertigo, visual phenomena
• Autonomic: Sweating, epigastric “rising” sensation
• Psychic: ” Deja vu,” affective changes ( eg, fear)
Focal Seizures
Etiology
·       Structural abnormality (eg, tumor)
·       Genetic epilepsy
·       Unknown
Clx
·       Origin: one cerebral hemisphere (Not lobe, hemisphere, and can affect consciousness)
·       Intact (simple) or impaired (complex) awareness
·       ± Secondary generalization
Dx
·        MRI of the 🧠
·       Electroencephalography

Headaches

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Migraine Tx
Abortives
• Triptans (eg, sumatriptan)
• NSAIDs (eg, naproxen)
• Acetaminophen
• Antiemetics (eg, metoclopramide, prochlorperazine)
• Ergots (eg, dihydroergotamine)
Preventatives
• Topiramate
• Valporate
• TCA
• BB (eg, propranolol)

A child w/ headache:
 Neurological sx:
No neurological sx?
·       Any child who has headache and the presence of seizures, sudden
worsening of headache, change in character of headache, or abnormal
neurologic examination must receive an imaging study of the brain to rule out
a structural lesion.
·       Headaches that are worse in the a.m. should suggest further investigation.
The posterior fossa may contain a mass lesion with minimal findings such as
ataxia.
Behavior management or biofeedback and self-hypnosis would be the principal means of treating a child who has stress headaches.

Tremors

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Tremors
Essential
·       ⓑ action tremor of the hands, usually without leg involvement
·       Possible isolated head tremor without dystonia
·       Usually no other neurologic signs
·       Relieved with alcohol in many cases
Parkinson’s disease
·       Resting tremor (4-6 HZ) that decreases With voluntary movement
·       Usually involves legs & hands
·       Facial involvement less common
Cerebellar
·       Usually associated with ataxia , dysmetria, or gait disorder
·       Tremor increases steadily as the hand reaches its target
Physiologic
·       Low amplitude (10-12 Hz)not visible under normal conditions
·       Acute onset with increased sympathetic activity (eg, drugs, hyperthyroidism, anxiety , caffeine)
·       Usually worse with movement & can involve the face & extremities

Nystagmus & Vertigo

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Common DDx of vertigo
Méniére Δ
·       Rx episodes lasting 20 minutes to several hours
·       SN hearing loss
·       Tinnitus and/or feeling of fullness in the 👂🏻
BPPV
·       Brief episodes triggered by head movement
·       Dix-Hallpike maneuver causes nystagmus
Vestibular Neuritis
·       Acute, single episode that can last days
·       Often follows viral syndrome
·       🆎 Head-thrust test
Migraine
·       Vertigo + Migraine (HA / Aura)
·       Sx resolve completely b/w episodes
Brainstem/
cerebellar stroke
·       Sudden-onset, persistent vertigo
·       Usually other neurologic

Nystagmus
Peripheral
Central
?
1.     BPPV
2.     Menière dz
3.     Vestibular neuritis
4.     Acoustic neuroma
1.     Stroke
2.     MS/Migraine
3.     CNS tumor
Nystagmus
1.     Horizontal ± vertical/torsinal
2.     <1 minute
3.     2-10 sec latency
1.     Purely vertical
2.     >1- minute
3.     No latency period
Postural
ⓝ
Unstable
Hearing loss or Tinnitus
yes
none
Other CNS
none
Usually present

Parkinson’s & Huntington’s

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Parkinson’s Dz
features
Idiopathic, slowly progressive
S/S
usual sx
Dx
Clx
Tx
There’re multiple agenst that can be used to tx Parkinson’s dz, they focus on: Dopamine.
1.     L-dopa + Carbidopa: ⤴️ dopamine in CNS, make pt as happy as possible, but may cause hallucination
2.     Bromocriptine: a dopamine agonist
3.     Antichoinergic (benzotropine): for tremor
4.     Amanditine: for mild cases, ⤴️ dopamine
5.     Seligiline, ❌ dopamine break down

aa61650e2a2856110861336386983eb6
Huntington disease
Clx
·       Motor: chorea, delayed saccades
·       Psychiatric: depression, irritability, psychosis, obsessive-compulsive symptoms
·       Cognitive: executive dysfunction
Dx
·       Genetic: AD, CAG trinucleotide repeat expansion
·       Neuropathology: loss of GABA-ergic neurons
·       Imaging: caudate nucleus and putamen atrophy
Tx
Tx: supportive
Can live: 10-20 years

Neuromuscular Disorders

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 Multiple Sclerosis
features
·       an idiopathic disorder exclusively of CNS (brain and cord) white matter.
·       Twice as common in 🙎🏻‍♀️s
·       a peak incidence at 20-40 years
S/S
·       Neuro sx (urinary incontinence, INO, optic neuritis, sensory/motor..)
·       Sexual function remains intact
·       scattered
·       Come and go
Dx
Start w/ MRI 
·       best dx modality 
·       multiple focal demyelination scattered in brain and spinal cord
·       asymmetric periventricular plaques
If MRI is unconclusive –> CSF analysis 
·       mononucleur pleocytosis
·       elevated CSF IgG (80%) 
·       oligoclonal bands on electrophoresis 
Tx
Acute exacerbation:
IV Steroids
Dz-modifying agents:
Interferon-ß
Glatimir
Sx-Specific
Fatigue: Amantadine
Spasticity: Baclofen or tizanidine
MS
Sx
·       Sensory disturbances
·       Motor weakness
·       Bowel or bladder dysfunction
·       Ocular: Optic neuritis (painful eye movement), internuclear ophthalmoplegia (impaired adduction on lateral gaze)
·       Lhermitte sign: Electrical sensation in limbs or back
·       Uhthoff phenomenon: Symptom worsening with ⤴️body temperature
RFs
·       Female, Caucasian,HLA-DRBI
·       Environmental: Location (USA, Europe), cold climate
·       Low vitamin D levels
·       Smoking
Dx
·       Episodic/progressive sx over time & space
·       Hyperintense lesions on T2-weighted MRI
·       Oligoclonal lgG bands on CSF analysis
Optic neuritis
#
·       Primarily in young female 👩🏻
·       Associated with MS
·       Immune-mediated demyelination
S/S
·       Acute, peaks at 2 weeks
·       Monocular vision loss
·       Painful eye movement
·       “Washed-out” color vision
·       Afferent pupillary defect
Dx
MRI of the orbits & brain
Tx
·       IV corticosteroids
·       35% of cases recur
—
 Myasthenia Gravis
Dx
·       1˚: Ach-Ab Test
·       The most accurate test is electromyography (EMG). The characteristic finding is a decremental ⤵️ in muscle fiber contraction on repetitive nerve stimulation.
When to use edrophonium challenge test? to ddx MG from cholenergic crisis.
Myasthina Crisis:
·       worsening sx: generalized + respiratory
DDx
·       Lambert-Eaton myasthenic syndrome (LEMS) 
·       muscle weakness that improves with use
·       autonomic manifestations
·       Botulism
·       poor or impaired pupillary response to light
·       EMG: a decremental ⤴️ muscle fiber contraction on repetitive nerve stimulation.
Tx
Acute flares:
1.     Plasmaphoresis / IVIG
2.     Corticosteroids
3.     Rituximab
Chronic:
·       Pyridostigmine
·       Steroids
·       Thymectomy
Myasthenia gravis
#
👩🏻: 20-30s
👨🏻: 60-80s
S/S
·       Fluctuating & fatigable proximal muscle weakness (Ocular, Bulbar, etc)
·       Worse during the day
Exacerbate
·       Drug: Abx: (FQ, Aminoglycosides) – NM blocking agents- BB, procainamide
·       Pregnancy/childbirth/infection
·       Surgery (especially thymectomy)
Dx
·       1° Ach Ab (SPECIFIC)
·       Bedside: Edrophonium (Tensilon)/ Ice pack test
·       CT Chest: thymoma
Tx
·       Acetylcholinesterase inhibitors (pyridostigmine)
·       Immunotherapy (CS, azathioprine)
·       Thymectomy
—
ALS
features
·       UMN + LMN affected, Anterior horn in Spinal Cord
·       most are sporadic (90%), others are familial
·       sensation is intact
·       bowel and bladder, sexual function, cognitive functions are not affected ✳️
S/S
·       asymmetric limb weakness (most common)
·       upper and lower motor neuron signs
UMN signs
·       spasticity or stiffness
·       hyperreflexia
·       dysarthria
·       dysphagia
LMN signs
·       atrophy
·       fasciculations
·       hyporeflexia
·       weakness
Dx
·       Clinical: Hx & PEx
·       Electromyelogram: widespread denervation and fibrillation potential
·       CSF + MRI ARE NORMAL
Tx
·       Riluzole: slow down progression
·       Otherwise –> supportive tx.
📝
Cause of death? Respiratory failure / recurrent aspiration penumonia
—
Guillain-Barré syndrome
Features
·       Immune-mediated demyelinating polyneuropathy
·       PMHx of GI/Resp Infection
S/S
·       Paresthesia, neuropathic pain
·       Symmetric, ascending weakness
·       ⤵️ DTR
·       Autonomic dysfunction (eg, arrhythmia, ileus)
·       Respiratory compromise
Dx
Clx
Supportive:
·       CSF: ⤴️ protein, ⓝ leukocytes
·       🆎EMG & nerve conduction
Tx
·       Monitor autonomic & respiratory function
·       IVIG /OR/pIasmapheresis