Tectal glioma with hydrocephalus presenting with spastic and ataxic quadriparesis.

Dear Sir,

The presentation of hydrocephalus in older children usually includes headache, vomiting, and papilledema.[12] The chronic symptoms of hydrocephalus in older children are ataxic and spastic cerebral palsy, hypothalamic and pituitary disturbance, mental retardation, specific learning disorders, chronic intermittent headache, behavioral changes, failure to thrive, and dizziness.[3] Presentation as isolated walking difficulty due to spasticity or ataxia in the absence of any other neurological symptoms and signs is rare. We report two patients with tectal glioma and obstructive hydrocephalus who presented with isolated gait difficulty.

This 9-year old boy presented with walking difficulty of 4 years duration due to stiffness of legs on a background of normal birth and developmental history. He reported worsening of his symptoms in last 1 year with difficulty in doing fine work with hands. On examination he had a head circumference (HC) of 55 cm, normal cranial nerves and fundus, hypertonia of all four limbs, brisk reflexes, and extensor plantars. MRI showed a tectal glioma with hydrocephalus [Figure 1a and b]. He underwent endoscopic third ventriculostomy (ETV). The intracranial pressure (ICP) was 15 mmHg.

Figure 1

MRI brain T2W sagittal acquisition: pre and postoperative images showing tectal glioma (arrow) and flow through floor of third ventricle (arrow). (a and b) first case, (c and d) second case

This 3-year-old girl presented with tremulousness of all four limbs of 1 year duration, and walking difficulty of 7 months, on a background of normal birth and developmental history. On examination she had a head circumference of 52 cm, normal cranial nerves, brisk stretch reflexes, extensor plantar response, and bilateral upper limb incoordination and gait ataxia. MRI showed tectal glioma with hydrocephalus [Figure 1c and d]. She underwent ETV. The intracranial pressure ICP was 15 mmHg. Both patients improved significantly at follow up.

Mild spasticity of lower limbs is not uncommon particularly in advanced cases of hydrocephalus.[4] However, isolated chronic walking difficulty due to spasticity or ataxia is an uncommon manifestation of hydrocephalus. The mechanism of gait disturbances in chronic hydrocephalus is stretching and distortion of paraventricular corticospinal tracts arising from leg area of motor cortex. These fibers have a longer distance to travel around the ventricles than those supplying the face and the upper limbs, hence vulnerable to stretch.[4]

The possibility of tectal glioma causing gait disturbances was also considered. Tectal gliomas most often initially manifest as hydrocephalus secondary to aqueductal stenosis. Most patients with tectal glioma have a long-standing history of headaches and on occasion “clumsiness" or ataxia.[2] In a series of nine children with tectal gliomas, all patients presented with clinical signs and symptoms of increased intracranial pressure secondary to hydrocephalus.[5] Though Parinaud's syndrome was observed in two patients, none had ataxia or spasticity.[5] From a review of literature it was concluded that the presenting clinical signs and symptoms in the vast majority of patients is related to hydrocephalus. Despite the tectal location and intrinsic nature of the tumors, cranial nerve palsies and long tract signs are surprisingly uncommon findings among these patients. The common focal neurologic deficits described in children with tectal gliomas are diplopia, visual deficits, and nystagmus.[56] Ataxia is an uncommon symptom in children with tectal gliomas. In a recent series of 44 children with tectal gliomas, balance problems and ataxia was present in 6 (13.6%).[7] However, isolated ataxia is rarely reported. In a series of 12 patients, only 1 patient presented initially with ataxia in the absence of signs of raised intracranial pressure.[6] The clinical outcome after treatment is sparingly described in the literature, most studies focused on radiological progression. In one series, all patients had good neurologic function after treatment.[6] There was no specific mention about improvement of ataxia. As both of our patients improved after ETV, we believed that hydrocephalus was the cause of gait disturbance rather than tectal tumor.