1. Neuropsychiatric Manifestation in 8 Years Old Girl Presented with Obstructive
Hydrocephalus Secondary to Posterior Fossa Tumour.
By: Muhamad Na’im B. Ab Razak
4th Year Medical Student, Universiti Sains Malaysia
8 Years old Malay girl who is the sixth out of seven siblings was referred from General Hospital
in view of obstructing hydrocephalus secondary to posterior Fossa tumor. Her father work as a
Disclaimer: Verbal permission given by relative to use picture as a medical education material to the author only.
labor and her mother is a housewife. Previously patient is well with normal milestone
with
development and able to go to school and follow the study syllabus. However, since one year
ago, patients frequently telling her mother about seeing ghost in which at that time she is
conscious, in objective space, involuntarily seeing the image, and without stimuli. The
hallucination become worsen since five months ago and the mother notice that patient talking to
the ghost and claims that she could hear the ghost speaking to her. Three months ago, she
develops bilateral lower limb wea
weakness but still be able to walk with assistance. Mother also
notices that patient keep on addressing herself as princess and easily irritable if mother ignores
her delusion. The weakness become progressive and she could not walk starting one month ago
.
and associates with headache and vomiting at morning. However, the parent only seek traditional
morning.
healer.
Therefore, author does not responsible if any party copy this for any purposes
Picture shows bilateral spasticity with decorticated posture.
Patient was brought to the hospital after her condition worsen in which her lower limb become
stiff, her head enlarge and she develop blurring of vision. On examination the head
examination,
circumference is 56.5 cm and tittubation is present. Cognitive function is intact. For the lower
limb, the entire joint is hypertonic, clonus, no muscle power and normal sensation. The reflexes
muscle
and Babinski sign could not be elicited. In the examination of the ears, patient could only see

2. hand waving which is approximately 5 cm distance and responding to light, papilloedema,
light
normal gaze, no nystagmus and eye reflex is normal. Examination of other part of body and
system is unremarkable. CT scan shows a tumor of the posterior fossa which suggests
differential diagnosis of medulloblastoma or ependymoma. There is also marked hydrocephalus
noted. Parent then gives consent for the placement of the ventriculo peritoneal shunt however
en ventriculo-peritoneal
refuses the resection of the posterior fossa tumor.
Right: round and well circumscribed enhanced lesion at the central of cerebellum, possibly arise
from fourth ventricle. Left: dilated lateral ventricle
. ventricle.
Discussion
Posterior fossa tumor account for 60% of all childhood tumor and the most common types of
tumor in children younger than 16 years old of age. It is usually near to the fourth ventricle and
alters the cerebrospinal fluid pathway leading to non-communicating, obstructive hydrocephalus.
Tumors of posterior fossa include medulloblastoma, pilocytic astrocytoma, ependymoma,
edulloblastoma, ependymoma
gangliocitomas, pineoblastoma, mixed gliomas, choroid plexus papilloma, haemangioblastoma
aemangioblastoma
and primitive neuroectodermal tumors (PNETs)
(PNETs).
Tumors in the posterior fossa are considered critical brain lesions. This is, primarily, because of
rs
the limited space within the posterior fossa and the potential involvement of vital brain stem
nuclei. Some patients should undergo an emergency operation, especially if they present with
especially
acute symptoms of brain stem involvement or herniation. [Hassan Ahmad Hassan Al Al-Shatoury]

3. The onset of symptoms in posterior fossa syndrome was diagnosed in less than 3 months in 37
(58%) patients, from 3 to 6 months in 9 (14%) patients, and after more than 6 months in 18
(28%) patients. [Ricardo Santos de Oliveira et al]
In a retrospective study made by Ricardo Santos de Oliveira et al, signs and symptoms of
posterior cranial fossa includes headaches (89%), ataxia (61%), papilloedema (41%), vomiting
(77%), cranial nerve palsy (III, IV and VI) (28%), motor deficits (11%), full anterior fontanelle
in infants, and torticolis (27%). 70 to 80% patient with posterior fossa tumor often reported with
the present of obstructive hydrocephalus and it often resulting in clinical deterioration of the
patient at the time of diagnosis.
Other signs and symptoms of posterior fossa tumor includes vermian syndrome, truncal ataxia,
falling tendency, wide base gait, limb ataxia, dysmetria, nystagmus, strabismus, meningismus
and macrocephaly.
Hydrocephalus may result from either over-production of CSF fluid, impaired absorption or
disruption of CSF outflow from the choroid plexus to the lateral ventricle, then to the
interventricular foramen of Monro, the third ventricle, the cerebral aqueduct of Sylvius, the
fourth ventricle, the 2 lateral foramina of Luschka and 1 medial foramen of Magendie, the
subarachnoid space, the arachnoid granulations, the dural sinus, and finally into the venous
drainage [Alberto J Espay]
If production of CSF exceeds absorption, then it will result in increase in ICP. As ICP increase,
temporal and frontal horns dilate first, often asymmetrically. This may result in elevation of the
corpus callosum, stretching or perforation of the septum pellucidum, thinning of the cerebral
mantle, or enlargement of the third ventricle downward into the pituitary fossa (which may cause
pituitary dysfunction. [Alberto J Espay]
In this patient, there is a difficulty in walking secondary to spasticity. It affects the lower limbs
preferentially because the periventricular pyramidal tract is stretched by the hydrocephalus.
[Alberto J Espay]
Psychiatric manifestation is common as long-term sequelae after posterior fossa tumor resection
during childhood but not during the disease manifestation. According to Maja Steinlin et al,
among the post tumor resection sequale of neuropsychiatric disorder includes behavioral deficits,
attention deficit problems, mutism, addiction problems, anorexia, uncontrolled temper tantrums
and phobia. Vermis involvement was related to an increase in neuropsychological and
psychiatric problems.

4. If it present during disease manifestation, is not commonly associated primarily with posterior
fossa tumor but rather arise due to brain changes as a result of intracranial mass that interfere
with various chemical and neuronal pathway of the brain. However, there are reported cases
involving neuropsychiatric presentation in patient with posterior fossa tumor.
Clara Limbäck Stokin et al has reported a case of haemangioblastoma localized in the fourth
ventricle that manifested with an unusual clinical picture featuring predominant psychiatric
symptoms and depression that went unrecognized until the patient's death.
Lea Pollak et al has reviewed seven cases with posterior fossa abnormalities presenting with
neuropsychiatric symptomatology. Seven patients (pts) (comprising 4 men and 3 women with
mean age 22 years) were diagnosed as suffering from psychosis (2 pts), major depression (1 pt),
personality disorders (2 pts) and somatoform disorders (2pts) (DSM-IV criteria). Brain CT scan
(7 pts) and MRI (4 pts) revealed tumors of the posterior fossa (2 pts), megacisterna magna (2pts)
and Dandy-Walker variant (2 pts). In one patient a fourth ventricle tumor was removed in
childhood
Theory lead to the development of neuropsychiatric disorder during the disease manifestation
includes derangement in the balance of dopamine, serotonin and noradrenergic networks has
been implicated in the pathogenesis of schizophrenia, affective and even personality disorders.
Disruption of the cerebellar output to mesial dopaminergic areas, locus coeruleus and raphe
nuclei, or deafferentation of the thalamolimbic circuits by a cerebellar lesion may lead to
behavioral changes. [Lea Pollak et al]
Reference
1) Alberto J Espay, "Hydrocephalus", eMedicine
http://emedicine.medscape.com/article/1135286-overview
2) Anna Clerico, Alessia Sordi, Giuseppina Ragni and et al, "Transient Mutism Following Posterior Fossa
Surgery Studied by Single Photon Emission Computed Tomography (SPECT)", Medical Pediatric Oncology
2002;38:445–448
3) Clara Limbäck Stokin, Damjan Glava & Mara Popovic, "A case of haemangioblastoma of the fourth
ventricle presenting with depression", BMJ Case Reports 2009
4) Hassan Ahmad Hassan Al-Shatoury, "Posterior Fossa Tumors", eMedicine
http://emedicine.medscape.com/article/249495-overview
5) Lea Pollak, Colin Klein, Jose Martin Rabey et al, " Posterior Fossa Lesions Associated with
Neuropsychiatric Symptomatology", International Journal of Neuroscience, Vol. 87, No. 3-4, Pages 119-
126, 1996
6) Maja Steinlin, Sara Imfeld, Prisca Zulauf and et al, "Neuropsychological long-term sequelae after posterior
fossa tumour resection during childhood", Brain, Guarantors of Brain 2003
7) Ricardo Santos de Oliveira et al "Hydrocephalus in posterior fossa tumors in children. Are there factors
that determine a need for permanent cerebrospinal fluid diversion?", Children Nervous System 24:1397-
1403, Springer-Verlag 2008