Neurology
Absence (petit mal) seizures
⁃
He is always daydreaming.
⁃
EEG during hyperventilation shows generalized,
symmetrical 3-Hz spike-and-wave activity on a normal background.
+ most appropriate
to treat the boy's disorder?
Valproic acid
+ Drugs of choice : Ethosuximide and valproic
acid.
+ A typical
absence seizure is characterized by brief (i.e., typically lasting for a
few seconds) periods of impaired consciousness.
⁃
do not have a post-ictal state.
⁃
EEG pendant Hyperventilation ==> reveals a
generalized 3Hz spike-and-wave pattern on a normal background.
Differential
:=========
+ A complex
partial seizure is characterized very like in absence seizures , but
⁃
have post-ictal confusion.
⁃
EEG pendant Hyperventilation ==> reveals a
Normal .
Acute glaucoma
Suspect acute glaucoma in a
patient with a
- sudden onset of eye pain,
- photophobia, and a
- mid-dilated pupil.
+ Tonometry is the best
diagnostic test.
Brown-Sequard syndrome
Brown-Sequard syndrome is Damage
to right-sided lateral spinothalamic tracts at T10.
associated with damage to
the lateral spinothalamic tracts, causing contralateral loss of pain and
temperature sensation beginning two levels below the level of the lesion (remember
that the spinothalamic tracts cross very early on in the spinal cord).
Therefore, a lesion of the right-sided lateral spinothalamic tracts at T1 0
will result in a left-sided loss of pain and temperature sensation beginning at
T12.
Cluster headache
+ Finding :
⁃
severe pain 'behind the left eye' which woke him up in
the middle of the night
⁃
pain intense like stabbing
+Tm => 100%
oxygen
Creutzfeldt-Jakob disease
Creutzfeldt-Jakob
disease is a fatal neurodegenerative disease caused by prions.
+ Suspect this
condition in an old patient (between 50- to 70-years-old) with
⁃
rapidly progressive dementia,
⁃
myoclonus and
⁃
periodic high voltage complexes on EEG.
+
Electroencephalogram (EEG) pattern of periodic synchronous bi or triphasic
sharp wave complexes is very typical.
+ Brain biopsy
shows cortical spongiform changes. The CSF is usually normal. Most cases are
sporadic (80-85%), others are familial or iatrogenic. Death usually occurs within 12 months.
+ There is no
specific treatment.
Dejerine-Roussy syndrome
Thalamic stroke
(Dejerine-Roussy syndrome). This condition is caused by a stroke involving
ventral postero-lateral (VPL) nucleus of the thalamus, which transmits sensory
information from the contralateral side of the body.
+ The classic
presentation involves
⁃
Contralateral hemianesthesia that can be accompanied
by transient hemiparesis, athetosis, or ballistic movements.
⁃
Dysesthesia of the area affected by the
sensory loss is characteristic, and is called thalamic pain phenomenon.
+ dysaesthesia dɪsɪsˈθiːzɪə/
nounMEDICINE
noun: dysesthesia
an abnormal unpleasant sensation felt when touched, caused by damage to
peripheral nerves.
Delirium
Delirium is an acute
confusional state due to organic illness which is commonly superimposed upon
dementia in the elderly. Urinary tract sepsis and metabolic disturbances are
common precipitants in older patients. The initial work-up of delirium should
include urinalysis and measurement of serum electrolytes.
Guillain-Barre syndrome (GBS)
The classic
presentation includes
⁃
ascending paralysis (weakness ) both bilateral
hands ad legs / Only lowers —> symetry
,
⁃
areflexia and sensory changes 3-4 weeks after
an upper respiratory tract infection or gastroenteritis.
+ LCR : Increase
Protein and Others are normal
+ Treated with : IV
immunoglobulin therapy and
plasmapheresis
+Which of the
following organisms is involved in the pathogenesis of this disorder?
=> Campylobacter
jejuni
Heat stroke
+ Finding :
⁃
Excercise under the sun and then fainted
⁃
Patient’s temperature > 40.5 C
+ Pathophysiology of his
current condition?
=> Failure
of thermoregulatory center
Huntington's
disease
This autosomal
dominant condition affects both sexes equally. The age at presentation is
typically between 30-50 years. The typical presenting features are
⁃
Mood/behavioral disturbances (depression, apathy),
⁃
Dementia, and
⁃
Choreiform(movement) movements (facial
grimacing, ataxia, dystonia, tongue protrusion, writhing movements of
extremities , sudden, jerky and irregular movements of his upper
extremities).
⁃
And Family history of similar symptoms.
+ Which of the
following is a typical CT finding in such patients?
=> Atrophy
of the caudate nucleus
Kaposi sarcoma
+ Kaposi sarcoma in HIV
patients is caused by human herpesvirus 8.
+ Cle : HIV + Papule brown
/ violet
+ The cutaneous lesions of
Kaposi sarcoma are asymptomatic, elliptical, and arranged linearly. Commonly
involved regions include the legs, face, oral cavity, and genitalia. The
lesions begin as papules, and later develop into plaques or nodules. The color
typically changes from light brown to violet. There is no associated necrosis
of the skin or underlying structures. In the US, this disease is most commonly
seen in homosexual HIV patients.
Lewy body dementia
is characterized by
⁃
Dementia ,
⁃
Visual hallucinations and
⁃
Motor features of Parkinsonism.
Multifocal leukoencephalopathy (PML)
Progressive multifocal
leukoencephalopathy (PML) is an opportunistic infection seen in
immunocompromised patients. It is caused by the JC virus, a human polyomavirus.
The exact mode of transmission is unknown. PML predominantly involves the
cortical white matter, but the brainstem and cerebellum may also be involved.
The lesions typically do not produce a mass effect, and the onset of symptoms
is usually gradual. The most common presenting symptoms are hemiparesis and
disturbances in speech, vision and gait. Cranial nerve deficits may
occasionally develop. An immunocompromised patient with focal neurological
deficits should raise the suspicion for PML, and this diagnosis is best
confirmed with MRI. Classic MRI findings in PML consist of multiple
demyelinating, non-enhancing lesions with no mass effects. There is no
effective treatment for PML, and the mean duration of survival from the time of
diagnosis is six months.
Myasthenia gravis.
Myasthenia gravis : is
pathology on neuromuscular junction (
post-synapse ) and is mediated by auto-antibodies against the acetylcholine
receptor.
The typical presentation
includes
⁃
fatigability and weakness of the skeletal
muscles. There are no reflex, sensory or coordination abnormalities. A
characteristic feature is that the symptoms are typically seen on exertion and resolve
with rest.
⁃
Weakness of the eye muscles can lead to
diplopia and ptosis, especially on looking over one's head. Abnormal eye
muscle movement, which cannot be attributed to one nerve or muscle, may be seen
on examination; however, unlike botulism, the pupils are always spared in MG.
Bulbar muscle involvement can lead to "myasthenic snarl", nasal
speech, and difficulty in chewing or swallowing. Myasthenia gravis is an
autoimmune disorder. The basic pathology lies at the neuromuscular junction and
is mediated by auto-antibodies against the acetylcholine receptor, leading to a
decrease in the number of available acetylcholine receptors (AchRs) at the
neuromuscular junctions. The histological hallmarks include decreased numbers
of acetylcholine receptors, simplification of the postsynaptic clefts, and
widening of the synaptic space with a normal presynaptic nerve terminal.
Tm symptoms : Oral anticholinesterase ( Treatment
with pyridostigmine )
+ Which of the following is
the best treatment to induce remission and provide long-term benefits in this
patient?
=>Thymectomy
(not choose Pyridostigmine)
Neurofibromatosis
There are several
neurocutaneous syndromes, and the most well-known of these is
neurofibromatosis.
+ Type 1
neurofibromatosis is characterized by cafe-au-lait spots, axillary
freckles, Lisch nodules of the iris, neurofibromas, and bony lesions.
+ Finding (2).
cafe-au-lait spots, , feeding problems, short stature, and learning
disabilities.
Patients may later
develop fibromas, neurofibromas or different tumors.
+ Type 2
neurofibromatosis is associated with brain tumors, especially
bilateral acoustic neuromas ( signe
of hearing loss….
)and cafe-au-lait spots.
+ MRI
with gadolinium ==> Recherché Acoustic
neuromas
Normal pressure hydrocephalus (NPH)
+ Clinic Traide :
⁃
abnormal gait,
⁃
dementia, and
⁃
urinary incontinence.
+ The best initial
treatment for this patient?
=> Haloperidol
Pick's disease
+ is a fronto-temporal
dementia.
+ The characteristic
features of this condition are
⁃
Personality changes (e.g., stopping from normal
hobbie, euphoria, disinhibition, apathy),
⁃
Compulsive behaviors (e.g., peculiar eating habits,
hyperorality), and
⁃
Impaired memory ( memory loss ).
⁃
Visuospatial functions usually remain intact. A
positive family history may be present.
Pseudodementia
+ is elderly patients with
dementia in the setting of severe depression.
+Finding :
⁃
dementia
⁃
Signed depression ( lost interest… )
⁃
CT scan normal
=> Antidepressants are
the treatment of choice.
Pseudotumor cerebri
(idiopathic intracranial hypertension)
ការមាន
hydrocephaly ដោយគ្មានមូលហេតុ
⁃
signe HTIC
⁃
CT scan / IRM normal.
Next step ?
=> Lumbar
ponction
Lumbar ponction
normal ==> Treatment with acetazolamide
+ Cause :
⁃
Medication side effect
: lsotretinoin
+ Complications is
likely to develop if this patient is left untreated?
=> Blindness
Shy-Dragger syndrome
Shy-Dragger syndrome, which
is a degenerative disease characterized by the following:
1 . Parkinsonism
2. Autonomic dysfunction (postural hypotension,
abnormal sweating, disturbance of bowel or bladder
control, abnormal
salivation or lacrimation, impotence, gastroparesis, etc.)
3. Widespread neurological
signs (cerebellar, pyramidal or lower motor neuron)
+ Always consider
Shy-Dragger syndrome when a patient with Parkinsonism experiences orthostatic
hypotension, impotence,
incontinence, or other autonomic symptoms. The accompanying bulbar dysfunction
and laryngeal stridor may
be fatal.
+Tm:
Anti-Parkinsonism drugs are
generally ineffective, and treatment is aimed at intravascular volume expansion
with fludrocortisone, salt supplementation, alpha-adrenergic agonists, and
application of constrictive garments to the lower body.
Strokes
Lacunar strokes
Lacunar strokes -
one of stroke that only affect
⁃
pure motor dysfunction
⁃
pure sensitive dysfunction
+ Cause of this
patient's symptoms?
=> Small vessel
hyalinosis
+
Middle ===>
Stroke only on face
Middle ===>
Proportionel
+ Anterior : =>
weakness lower > upper extremity
+ Right parietal
cortex ==> Hemi-neglect syndrome
left
+ Internal
capsule involvement ==> unilateral motor hemiparesis of the face, arm
and leg without any higher cortical dysfunction and visual field abnormalities
+ Left cortical infarct => hemiparesis
+ Dysphasia ( can’t speak )
Subarachnoid hemorrhage
This patient has
subarachnoid hemorrhage. "Cerebral salt-wasting syndrome" may occur
in patients with subarachnoid hemorrhage.
The pathology involves:
(1) an inappropriate
secretion of vasopressin, which causes water retention, and
(2) an increased secretion
of atrial/brain natriuretic peptide, which causes cerebral salt-wasting.
These changes result in
hyponatremia, which usually resolves within 1-2 weeks. SIADH is also commonly
seen in patients with intracranial hemorrhage. SIADH also results in
hyponatremia, for which
water restriction is the initial treatment of choice.
Syndrome de la queue de
cheval ( Cauda equina syndrome )
Cauda equina syndrome is
acute symptoms of spinal cord compression.
⁃
absent rectal tone,
⁃
urinary incontinence,
⁃
motor and sensory loss in the extremities
This is a surgical
emergency, and a neurosurgeon should be notified as soon as
Syringomyelia (Cord
cavitation)
The above patient is most
likely suffering from syringomyelia. Areflexic weakness in the upper
extremities and dissociated anesthesia (loss of pain and temperature with
preserved position and vibration) in a "cape" distribution are
classic findings of this condition.
The pathology involves
cavitary expansion of the spinal cord, which may produce myelopathy. There is
destruction of the gray and white matter adjacent to the central canal. The
most characteristic feature is the presence of a cord cavity, which usually
communicates with the central canal of the spinal cord. The most frequent site
of involvement is the lower cervical or upper thoracic region. When the
syringes occur in the upper cervical cord and extend proximally to involve the
medulla oblongata, the condition is called syringobulbia. Acquired causes of
syringomyelia include trauma, inflammatory spinal cord disorders or spinal cord
tumors. Occasionally, syringomyelia is idiopathic.
Tick-borne paralysis
Tick-borne paralysis ,
Patients usually present with progressive ascending paralysis over hours to
days. Fever is typically not present; hence, a history of fever or prodromal
illness makes the diagnosis unlikely. Sensation is usually normal. The CSF
examination is typically normal.
+ Meticulous search
and removal of the tick usually result in improvement within an hour and
complete recovery after several days.
Trigeminal neuralgia
Trigeminal
neuralgia is characterized by pain in the distribution of the branches of the
fifth nerve. The diagnosis is generally clinical and based on the
characteristic
⁃
pain,
which is severe, intense, burning or electric shock-like. or pain as knife-like
⁃
This symptom occurs in paroxysms that last a
few seconds to minutes each, but occurs many times a day.
⁃
Carbamazepine
is the drug of choice.
======= careful :
Multiple
sclerosis in a young female with bilateral trigeminal neuralgia.
Waterhouse-Friderichsen syndrome
The Waterhouse-Friderichsen
syndrome is acute adrenal insufficiency caused by profound,
usually bilateral bleeding into the adrenal glands because of disseminated
intravascular coagulation.
The Waterhouse-Friderichsen
syndrome (adrenal glands hemorrhage) is a very serious complication of
Meningococcal meningitis caused by Neisseria meningitidis.
Wernicke's encephalopathy
Wernicke's encephalopathy :
+ Cause => defiency
Vitamin B1 ( all alcoholic and/or malnourished patients )
+ Triad of
⁃
encephalopathy (confusion , altered mental statue)
⁃
oculomotor dysfunction ( Nystagmus , conjugate gaze
palsy , pupil dilated … )
⁃
gait ataxia ( gait instability )
+ Tm => Administration
of thiamine
