NEUROLOGY

CLINICAL PEARLS

Kevin Rey B. Basco, RMT, MD

MUST KNOWS!

• NEURODEGENERATIVE DISEASES
 PARKINSON DISEASE
 HUNTINGTON DISEASE
 ALZHEIMER DISEASE
 CREUTZFELDT-JAKOB DISEASE
 AMYOTROPHIC LATERAL SCLEROSIS

• NEUROCUTANEOUS DISEASES
 TUBEROUS SCLEROSIS
MUST KNOWS!
• DEMYELINATION/AUTOIMMUNE
MULTIPLE SCLEROSIS
MYASTHENIA GRAVIS
GUILLAIN-BARRÉ SYNDROME

• WERNICKE-KORSAKOFF SYNDROME

• INTRACRANIAL HEMORRHAGE
EPIDURAL
SUBDURAL
SUBARACHNOID
MUST KNOWS!

• VISUAL FIELD DEFECTS

 ANOPIA
 BITEMPORAL HEMIANOPIA
 HOMONYMOUS HEMIANOPIA

• APHASIA
 BROCA
 WERNICKE
 GLOBAL
NEURODEGENERATIVE DISEASES
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test
PARKINSON Degeneration of Parkinson  Vascular  Clinical diagnosis  Levodopa
dopaminergic TRAPS your dementia  A good response to plus
“Deficiency of neurons in the SNc body:  Huntington levodopa Carbidopa
Dopamine”  Tremor (at  Alzheimer MOA:
rest)  Protein aggregates: combination
 Rigidity Tau, a-synuclein with a peripheral
(cogwheel) decarboxylase(C
 Akinesia  Lewy body arbidopa)
(or (diagnostic finding) inhibitor to
bradykines prevent its
ia) peripheral
 Postural metabolism to
instability dopamine
 Shuffling
gait

HUNTINGTON Autosomal dominant Chorea,  Parkinson  Clinical diagnosis 

trinucleotide (CAG) athetosis,  Alzheimer  Genetic testing Tetrabenazin
 “Increase Dopamine” repeat expansion in aggression,  FTD  Biopsy: e
the huntington (HTT) depression, Intraneuronal inclusions MOA: Inhibit
gene on dementia containing aggregates vesicular
chromosome 4 (sometimes of ubiquitin and the monoamine
initially mutant protein transporter
 Inc.dopamine, mistaken for huntington (VMAT)
 Dec.GABA & ACh in substance dopamine
brain abuse) Protein aggregates:   vesicle
Polyglutamine packaging and
aggregates release
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test

ALZHEIMER Accumulation of Memory  FTD  Clinically:  Donepezil

two proteins (Aβ impairment  CJD Diagnosis of (10mg OD)
and tau) and exclusion  Rivastigmin
progressing e (9.5-mg
Asso. w/ the ff to language  Biopsy: Neuritic patch daily)
altered proteins: and plaques and or ( 6mg
visuospatial Neurofibrilllary BID)
ApoE-2:  risk of deficits, tangle (NFTs) MOA: AChE
sporadic form followed by inhibitors
executive  Aβ, Tau protein
ApoE-4:  risk of dysfunction aggregates
sporadic form
APP, presenilin-1 Pneumonia:
usual
terminal
event
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test
AMYOTROPHIC Cause of sporadic Combined  Multifocal  Primarily Clinical  Riluzole
LATERAL ALS is not well UMN & LMN motor  Pathology: (produces a
SCLEROSIS (ALS) defined degeneratio neuropathy Loss of these modest
n with BUNINA bodies lengthening
a.k.a “ Lou Gehrig Can be cause by conduction of
disease” defect in  Difficulty block  Protein survival)
superoxide walking (MMCB) aggregates: MOA:
dismutase 1  Tripping/F TPD-43, FUS decrease
alling neuron
Abundant  Weaknes  Autopsy findings: glutamate
GLUTAMATE s hands, Thinned-out anterior excitotoxicity
legs, horn neurons, CN
feet, motor nuclei, &  Still no cure
ankles corticospinal tract
 Slurred degeneration
speech
 Dysphagi
a
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test

Creutzfeldt- Rapidly  Dementia  Histology:  no known

Jakob disease Prions (PrPc to progressive with Lewy spongiform effective
PrPsc sheet [β- (weeks to bodies degeneration and therapy
pleated sheet months)  Alzheimer astrocytic gliosis
resistant to dementia  FTD
proteases]) with  conformation-
myoclonus dependent
(“startle immunoassay
myoclonus”) (CDI)
and ataxia.

Commonly
see periodic
sharp waves
on EEG and
 14-3-3
protein in
CSF
TUBEROUS SCLEROSIS (NEUROCUTANEOUS)
Pathogenesis Clinical Differential Diagnosis/ Treatment
Features Diagnosis Lab Test
HAMARTOMA  Glioblastoma  Genetic testing  Sirolimus
TSC1 (hamartin) S Muliforme  CT or MRI
mutation on Hamartomas  Hydrocephalus
chromosome 9 in CNS and
or skin;
TSC2 (tuberin) mutation Angiofibromas
on chromosome 16 ; Mitral
regurgitation;
Ash-leaf spots;
cardiac
Rhabdomyom
a; (Tuberous
sclerosis);
autosomal
dOminant;
Mental
retardation
(intellectual
disability);
renal
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test

MULTIPLE Autoimmune Sensory  Parkinsonis  Clinical  β-

inflammation and loss Optic m  Increase IgG level interferon,
SCLEROSIS demyelination of neuritis  Chorea and myelin basic glatiramer,
CNS (brain and Weakness  Isolated protein in CSF natalizuma
spinal cord) with Paresthesia dementia  Oligoclonal bands b (Stop
subsequent are diagnostic relapses
axonal damage Symptoms  MRI is gold and
may standard: halt/slow
Most often affects exacerbate Periventricular progression
women in their with plaques (areas of )
20s and 30s increased oligodendrocyte  IV steroids
body loss and reactive (acute
temperatur gliosis) flare)
e (eg, hot
bath,
exercise)
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test

Dec. AChRs at Proximal/As  Congenital  Anti-AChR  Pyridostig

NMJ due to an Ab- ymmetrical Myasthenic antibodies mine
MYASTHENIA mediated limb Syndrome  Antibodies to MOA:
GRAVIS autoimmune weakness &  Lambert- MuSK Anticholineste
attack by 3 fatigability Eaton  Edrophonium test rase w/c
mechanisms: of muscles Myasthenic inhibits
 Accelerated worsens Syndrome metabolism o
turnover of later in the  Multiple f ACh
AChRs day Sclerosis
 Damage to the  Dermatomy
postsynaptic Diplopia & ositis
muscle ptosis
membrane “snarling”
 Blockade of expression
the active site
of the AChR Ass. w/
Thymoma
Disease Pathogenesis Clinical Differential Diagnosis/ Lab Treatment
Features Diagnosis Test

GUILLAIN- Complement *Areflexic  Acute  Histology:  High dose

deposition along *Bulbar Myelopathy perivenular and Immunoglo
BARRÉ the outer surface weakness  Chronic endoneurial bulin (IVIg)
SYNDROME  of the Schwann *Dysphagia inflammator infiltration of the
cells *Dysarthria y lymphocytes,  Plasmapher
demyelinati macrophages and a e-sis
Ass. w/ history of *Respirator ng few plasma cells
infection y muscle polyneuropa
(Campylobacter paralysis thy  CSF analysis:
jejuni, CMV, Acellular with an
Influenza virus) *Symmetric elevated protein
Ascending levels
muscle
weakness/
paralysis
 WERNICKE-KORSAKOFF SYNDROME
PARAMETER WERNICKE KORSAKOFF
ENCEPHALOPATHY SYNDROME
Syndrome  Psychotic symptoms or  Disturbance of short
ophthalmoplegia term memory &
 Memory device confabulation
(Ataxia, confusion,  Memory device
ophthalmoplegia) (Confabulation,
hallucination,amnesia)
Duration  Acute  Chronic

Reversibility w/ Thiamine  Yes  No

Morphology  Hemorrhage & necrosis  Cystic space w/

of the mamillary bodies hemosiderin-laden
and the walls of the 3rd macrophages in
& 4 ventricles
th
dorsomedial nucleus of
the thalamus
 INTRACRANIAL HEMORRHAGE
PARAMETER EPIDURAL SUBDURAL SUBARACHNOID
HEMATOMA HEMATOMA HEMORRHAGE
Age/Onset Middel age Extremes of age Older population

Clinical picture RAPID neurologic GRADUAL Acute severe

symptoms w/ neurologic headache
LUCID intervals symptoms, “Worst headache
delayed onset of my life”

Blood vessel Middle Meningeal Bridging/Emissary PCA & ICA

injured artery veins
CT scan finding LENTIFORM CRESCENT-
density SHAPED density
VISUAL FIELD DEFECTS

a. cutting the optic nerve causes blindness in

the ipsilateral eye
b. cutting the optic chiasm causes
heteronymous bitemporal hemianopia
c. cutting the optic tract causes homonymous
contralateral hemianopia
d. cutting the geniculocalcarine tract causes
homonymous hemianopia with macular
sparing
 APHASIA
TYPE SPEECH COMPREHENS
FLUENCY ION

Broca (expressive) Nonfluent Intact Broca = Broken

Speech

Wernicke (receptive) Fluent Impaired Wernicke is Wordy

but makes no sense.
Patients do not have
insight.

Global Nonfluent Impaired Arcuate fasciculus;

Broca and Wernicke
areas affected (all
areas)
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