Neuropsychiatric Manifestation in 8 Years Old Girl Presented with Obstructive Hydrocephalus Secondary to Posterior Fossa Tumour.

By: Muhamad Na’im B. Ab Razak

4^th Year Medical Student, Universiti Sains Malaysia

Disclaimer: Verbal permission given by relative to use picture as a medical education material to the author only. Therefore, author does not responsible if any party copy this for any purposes

Picture shows bilateral spasticity with decorticated posture

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 labor and her mother is a housewife. Previously patient is well with normal milestone 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 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 healer.


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 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 and Babinski sign could not be elicited. In the examination of the ears, patient could only see hand waving which is approximately 5 cm distance and responding to light, papilloedema, 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 refuses the resection of the posterior fossa tumor.

round and well circumscribed enhanced lesion at the central of cerebellum, possibly arise from fourth ventricle

dilated lateral 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, gangliocitomas, pineoblastoma, mixed gliomas, choroid plexus papilloma, haemangioblastoma and primitive neuroectodermal tumors (PNETs).

Tumors in the posterior fossa are considered critical brain lesions. This is, primarily, because of 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 acute symptoms of brain stem involvement or herniation. [Hassan Ahmad Hassan Al-Shatoury]

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.

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

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