Medicine PG poster presentation

1. 1
CRANIO VERTEBRAL JUNCTION ANOMALIES
REFERENCES : 1. ADAMS AND VICTOR’S PRINCIPLES OF NEUROLOGY9TH EDITION ;
2. J. Neurol. Neurosurg. Psychiat., 1949, 12, 61. DR.K.SHANKAR M.D.,(General Medicine)FINAL YEAR
The C-V junction is a transition site between mobile cranium and relatively rigid spinalcolumn.It is also the site of the medullo spinal
junction.
CV anomalies are defects of development,notnecessarilycongenitaland may not manifest at birth.
CLASSIFICATION
I. SKELETAL ANOMALIES II. NEUROAXIAL ANOMALIES III. COMBINED SKELETAL AND
NEUROAXIAL ANOMALIES
1. Platy basia
2. Basilar invagination
3. Occipitalisation of atlas
4. Klippel - Feil anomaly
5. Atlanto - Axial dislocation
1. Arnold-chiarimalformation
2. Dandy-walkersyndrome
3. Occipito cervical meningomyelocele
4. Cyst in posterior fossa
Often the anomalies are in different combinations and hence the difficulty in an appropriate terminology in every case. Certain
terms are conventionally used to express the anomalies, as follows
ANOMALY DEFECT CLINICAL PRESENTATION
PLATY BASIA
Angle > 143 degree ( 135-143 normal)
1. Flatteningof the base of the skull
2. The angle ( welcherbasal angle ) formed
by intersectionof the plane of clivusand
the plane of the anteriorfossa is greater
than 143 degree
Platybasia perse is notknownto produce any
neurological disorder,butitmaybe associated
withbasilarinvagination
BASILAR INVAGINATION ( IMPRESSION )
1. Upward bulgingof the occipital condyles
( whichbearsthe thrustof spine ) are
displacedabove the plane of foramen
magnum
2. May be congenital oraquired
1. Classical symptoms- occipital
headache,hyperalgesiainthe distribution
of secondcervical nerve roots
2. Characteristicshortnessof neck
3. Combinationof cerebellar/spinal signs
4. Paralysisof lowercranial
nerves/involvementof pyramidal
tract/cerebellumare common
5. Normal pressure hydrocephalusmay
develop
KLIPPEL - FEIL ANOMALY 1. Fusionof uppercervical vertebrae orthe
atlasto the occiput
2. ASSOCIATEDDISORDERS
a).scoliosis
b).urinarytractanomaly
c).abcence of auditorycanal
d).congenitalheartdisease(VSD)
e).synkinesia
f).facial asymmetry
g).webbingof neck
h).torticollis
i).sprengel’sdeformity
Feil’s triad
I) Lower posterior hair line
II) Short neck
III) Limitation of head and neck
movement

2. 2
CRANIO VERTEBRAL JUNCTION ANOMALIES
REFERENCES : 1. ADAMS AND VICTOR’S PRINCIPLES OF NEUROLOGY9TH EDITION ;
2. J. Neurol. Neurosurg. Psychiat., 1949, 12, 61. DR.K.SHANKAR M.D.,(General Medicine)FINAL YEAR
ANOMALY DEFECT CLINICAL PRESENTATION
ATLANTO AXIAL DISLOCATION
In atlantoaxial joint,the odontoidprocess(dens)is
completelyencircledbythe anteriorarchof atlas
ventrally,bythe transverse atlantal ligamentous
complex dorsallyandlateral massesof atlason
eitherside
Odontoinprocesscompletelyfuseswiththe body
of axis,thusdensbecomeslikea“buttonina
buttonhole”
The ligamentouscapsule ,around the jointare
loose & permitwide range of lateral androtatory
movementlike“wheel aroundaxle”
May be congenital oracquired
Acquiredcauses – a) trauma
b) tuberculosis
c) rheumatoid arthritis
d) ankylosingspondylosis
1. Suddenworseningafterminorinjuries
that flex head
2. Tendernessoveratlantoaxialregion
3. Restrictionof rotationof head
4. Absence of lowercranial nerve palsy
5. Marked spasticquadriparesiswithlossof
dorsal columnsensation
6. All DTR’sexaggerated
7. Plantarextensor
8. Absence of hydrocephalus
OCCIPITALISATION OF ATLAS
Characterised bypartial orcomplete fusionof the
bonyring of atlasto the base of the occiput
Patientmayhave
1. Torticollis
2. Short neck
3. Low hairline
4. Restrictedneckmovement
DANDY – WALKER SYNDROME
Failure of the foraminae of magendie&luschka
to open
The key features of this syndrome are
1. an enlargement of the fourth ventricle;
2. a partial or complete absence of
the cerebellar vermis, and
3. cyst formation near the internal base of
the skull
1. Symptoms in early infancy
a). slower motor development and
b). progressive enlargement of the
skull.
2. Symptoms in older children
a). increased intracranial pressure
b). signs of cerebellar dysfunction
3. Other symptoms
a). increased head circumference
b). bulging at the back of the skull
c). problems with the nerves that
control the eyes, face and neck
d). abnormal breathing patterns
ARNOLD – CHIARI MALFORMATION
It consist of
1. a downward displacement of the
cerebellar tonsils through the foramen
magnum (the opening at the base of the
skull)
2. sometimes causing non-
communicating hydrocephalus as a
result of obstruction of cerebrospinal
fluid (CSF) outflow
1. Headaches
2. Tinnitus
3. Dizziness and vertigo
4. Nausea
5. Nystagmus
6. Facial pain
7. Muscle weakness
8. Impaired gag reflex
9. Restless Leg Syndrome
10. Sleep Apnea
11. Dysphagia
12. Impaired coordination
13. Dysautonomia