Other CNS Disorders Flashcards
Acute demyelinating polyradiculoneuropathy (symmetric ascending muscle weakness or paralysis)
Paralysis usually ff a NONSPECIFIC VIRAL INFECTION (GIT or RT) by 10 days (Campylobacter jejuni and herpesvirus)
Guillain Barre syndrome
postinfectious polyneuropathy involving mainly motor
Etiology of Guillain Barre syndrome
Autoimmune reaction that develops in response to a previous infection – ABERRANT DEMYELINATION of PERIPHERAL NERVES and VENTRAL MOTOR NERVE ROOTS
Landry Ascending Paralysis
Weakness begins in the LOWER EXTREMITIES and progressively involves the TRUNK, UPPER LIMBS and BULBAR MUSCLES
Other physical findings associated with GBS
CRANIAL NERVE DEFICITS - dysphagia, dysarthria, facial weakness, papilledema, autonomic dysfunction, respiratory muscle paralysis
MILLER-FISHER SYNDROME - acute ophthalmoplegia, ataxia, areflexia
The last function to resolve in GBS
Extremity weakness
The last function to recover in GBS
Tendon reflexes
3 Clinical features are predictive of POOR OUTCOME with sequela
CN involvement
Need for intubation
Maximum disability at the time of presentation
Laboratory findings in GBS
INCREASED PROTEIN
normal glucose
(-) pleocytosis
ALBUMINOCYTOLOGIC DISSOCIATION - dissociation b/w high CSF protein
Management of GBS
admit - observation
IVIG - rapidly progressive ascending paralysis
supportive care
Methylprednisolone (high dose pulse (V) - relapses
headache, vomiting, decreased activity
new onset falling, stumbling gait
MEDULLOBLASTOMA
cerebellum
4-8 y/o
MALE
MC malignant brain tumor in children
Medulloblastoma
HOMER WRIGHT ROSETTES - circular patterns of tumor cells surrounding a center of neutrophils
Infratentorial Tumors
Cerebellar astrocytoma - MC; best prognosis
Medulloblastoma
Brain stem glioma
Ependymoma
Supratentorial Tumors
Craniopharyngioma
Optic nerve glioma
Astrocytoma
Choroid plexus papilloma
Strokes most often involved this artery
MCA
Diagnostic of choice in Arterial Ischemic Stroke (AIS)
MRI
3 main etiologies of Arterial Ischemic Stroke (AIS)
ARTERIOPATHY - disorders of cerebral arteries
CARDIAC - cardioembolic strokes
HEMATOLOGIC - sickle cell anemia (400 fold increased risk of stroke), IDA, coagulation disorders
Management of AIS
ANTITHROMBOTIC STRATEGIES
Heparin
Aspirin
NEUROPROTECTIVE STRATEGIES Glucose control Temperature control Seizure prevention Maintenance of cerebral perfusion
Secondary Stroke Prevention (antiplatelet therapy in arteriopathy and anticoagulation in cardiogenic causes)
Rehabilitation
Thrombotic occlusion of venous structures create increased ICP, cerebral edema and venous infarction or hemorrhage
Cerebral Sinovenous Thrombosis (CSVT)
Diagnostic of choice in Cerebral Sinovenous Thrombosis (CSVT)
Contrast CT venography or MR venography
Management of Cerebral Sinovenous Thrombosis (CSVT)
Anticoagulation therapy (unfractionated or LMWH)
The MCC of childhood subarachnoid and intraparenchymal hemorrhagic stroke
AV malformation
Arise from a DEFECT IN DIFFERENTIATIONOF THE PRIMITIVE ECTODERM
Neurocutaneous Syndrome
AD trait
Heterogeneous disease with a wide clinical spectrum varying
from severe MR & incapacitating seizures to normal intelligence and a lack of seizures, often within the same family
Affects many organs like skin, brain, heart, kidney, eyes, lungs, bone
Tuberous Sclerosis
Mutated genes in Tuberous Sclerosis
TSC1 – hamartin
TSC2 - tuberin
tumor suppressor genes
Characteristic brain lesions in Tuberous Sclerosis
TUBERS (subependymal region) – undergo calcification and project into the ventricular cavity – CANDLE-DRIPPING APPEARANCE
Clinical Manifestations of Tuberous Sclerosis
Infantile spasms
ASH LEAF
generalized seizure SHAGREEN PATCH (lumbosacral region) - roughened, raised lesion with an orange peel
SUBUNGUAL OR PERIUNGUAL FIBROMA
RHABDOMYOSARCOMA
BILATERAL ANGIOMYOLIPOMAS and CYTS
2 types of retinal lesions on tuberous sclerosis
mulberry tumors
round gray flay lesions
AD
every system may be affected
d.t. ABNORMALITY of NEURAL CREST CELL DIFFERENTIATION and MIGRATION during the early stages of embryogenesis
Neurofibromatosis
NF-1
NF-2
The most prevalent type of Neurofibromatosis
NF-1
diagnosed when any 2/7 of the ff are
- 6 or more CAFE AU LAIT MACULES >5mm in diameter in prepubertals & >15mm in postpubertal individuals: present at
birth & increase in size, number & pigmentation with predilection for the trunk & extremities with sparing of the face - AXILLARY OR INGUINAL FRECKLING consists of multiple hyperpigmented areas 2-3 mm in diameter
- 2 or more iris LISCH NODULES (hamartomas located within the iris)
- 2 or more NEUROFIBROMAS (along the skin, PNS, blood
vessels & within viscera) or one plexiform neurofibroma - Distinctive osseous lesion
- OPTIC GLIOMA
- 1st-degree relative with NF-1 whose diagnosis was based on the
aforementioned criteria - Majority of mutations in NF-1 occur in the paternal germline
- High incidence of learning disabilities
NF-2 diagnosis
1 of the ff is present:
- Bilateral 8th nerve masses (acoustic neuroma)
- Parent, sibling, or child with NF-2 & either unilateral 8th nerve masses or any 2 of the ff: neurofibroma, meningioma, glioma, schwannoma
NF-1
cafe au lait macules axillary or inguinal freckles lisch nodules neurofibromas osseous lesion optic glioma
Pattern of weakness in GBS
Ascending symmetric paralysis
MC location of brain tumors in children
INFRANTENTORIAL
MCC of arterial ischemic stroke in children
ARTERIOPATHY
Diagnostic of choice in cerebral sinovenous thrombosis
Contrast CT venography or MR venography
Etiology of Brain Abscess
embolization d.t. CHD w/ R > L shunts
meningitis
chronic OM and mastoiditis
face and scalp infections
orbital cellulitis
dental infections
penetrating head injuries
VP shunt infections
Brain Abscess
S. aureus
Streptococci
Anaerobes
Gram (-) aerobic bacilli (Proteus, Pseudomonas, Haemophilus, Citrobacter)
CEREBRUM (80%)
single
Diagnostic of Brain Abscess
Cranial CT scan and MRI
Indications of Surgery (Brain Abscess)
(+) gas in the abscess multiloculated abscesses posterior fossa location fungal cause associated infections - mastoiditis, periorbital abscess, sinusitis
