Correspondence Address:
Syed Ahmed Zaki
Department of Pediatrics, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai
India

Full Text

To the Editor,

Headache is a common problem in children. The effects of headache on the child's academic performance, personality and interpersonal rela­tionship depend on their etiology, frequency and intensity. The commonest cause of headache in an otherwise normal child is functional in origin. However, if it remains persistent, it may indi­cate a severe underlying disorder like brain tumor, sinus thrombosis etc. Thus, careful evaluation of children with recurrent, severe, progressive or unconventional headache is mandatory.

A 9-year-old male child with frequently re­lapsing nephrotic syndrome (FRNS), diagnosed when he was 6-years-old, was admitted with complaints of persistent headache of 15 days du­ration. The headache was of acute onset, gene­ralized, continuous and more in the frontal re­gion. There was no obvious aggravating or re­lieving factors. There was no history of any diurnal variation, oliguria, fever or vomiting. Mild periorbital puffiness was noticed two days prior to admission. For the above complaints, the child had received symptomatic treatment with­out any relief.

At presentation, he was on treatment with pred­nisolone (0.5 mg/kg/day) on alternate days along with calcium supplements. Past medical history was negative for migraine headaches, head trau­ma, febrile illness, substance abuse and vitamin intake. Family history was unremarkable. On examination, the child was conscious, alert and afebrile. Periorbital and mild pedal edema were present. He was normotensive with a respi­ratory rate of 28/min and pulse rate of 100/min. Cranial nerve examination was normal and there was no papilledema on fundus examination. Vi­sual acuity was normal. Superficial and deep tendon reflexes were normally elicited. Motor, sensory, and cerebellar examination were nor­mal. There were no signs of meningeal irrita­tion. Other systemic examinations were normal.

Blood investigations showed total leukocyte count of 10,200/mm 3 , hemoglobin of 8.4 gm/dL with packed cell volume of 28.2%, platelet count of 5.9 lakhs/mm 3 , serum creatinine of 0.6 mg/dL, serum albumin of 3.1 gm/dL, total pro­tein of 5.2 gm/dL and blood urea of 22 mg/dL. Serum electrolytes were normal and erythrocyte sedimentation rate was 60 mm during the first hour. Coagulation profile showed prothrombin time of 12 sec, partial thromboplastin time of 33 sec, activated protein C resist of 2.8% (nor­mal 2.3-4.6%), functional protein C of 87% (nor­mal 70-130%) and free protein S of 69% (nor­mal 65-140%). Urinary analysis showed neph­rotic range proteinuria (4+). Serum complement C3 and C4 were normal.

Persistent headache not responding to usual mea­sures prompted us to evaluate further. Contrast computed tomographic (CT) scan of brain was done which revealed filling defect in the straight sinus extending into the right transverse sinus suggestive of thrombosis. There was no cere­bral parenchymal or brainstem lesions [Figure 1].{Figure 1}

Treatment of cerebral venous thrombosis was started immediately with administration of intra­venous heparin. A loading dose of low molecu­lar weight heparin of 150 IU/kg subcutaneously was given followed by 100 IU/kg of heparin twice daily. Daily infusion of fresh frozen plas­ma (FFP) as a source of anti-thrombin III (AT III) was also started. The child was started on relapse regimen of the nephrotic syndrome with prednisolone at 2 mg/kg/day in divided doses. He went into remission after five days and gra­dually made a complete clinical recovery over one week. Warfarin was added and the dose of heparin was gradually decreased. Heparin admi­nistration was stopped after seven days and the patient was discharged on warfarin. There was no neurological impairment on discharge. CT scan brain was repeated after one month which showed reduction in the thrombosis. The child has been followed-up and remains asympto­matic seven months later.

Evaluation of headache in the context of underlying disease and associated symptoms is important as it can give clues regarding the etiology. Headache due to increased intracranial pressure may be worse in the morning and improve as the day progresses; also, it gets aggravated by sneezing, coughing, or straining. Also, certain underlying medical conditions like the nephrotic syndrome predispose a child for sinus thrombosis leading to headache.

Thrombotic complications of the nephrotic syn­drome are related to hypercoagulable states and the possible mechanisms for this state include:



loss of body's own anticoagulant factors like plasminogen, antithrombin III, protein C and protein S,enhanced production of clotting factors like factor V, combined factor VII, X and fibri­nogen,hypercholesterolemia, andthrombocytosis and platelet hyperactivity. [1]

The other contributory factors are circulating immune complexes, corticosteroids, diuretics, volume depletion, immobilization and hypoal­buminemia.

Cerebral venous sinus thrombosis (CVST) in children with the nephrotic syndrome is unco­mmon. [2],[3] Patients may have generalized or focal neurologic signs and symptoms. They may pre­sent with headache, nausea, vomiting or sei­zures. The signs and symptoms of cerebral ve­nous thrombosis are occasionally non-specific as in our patient making the clinical diagnosis difficult. Hence, a high degree of suspicion is required and the diagnosis of CVST should be considered in any patient with the nephrotic syndrome who has persistent headache.

In the presence of an appropriate clinical his­tory, cranial CT provides an excellent screening procedure. However, magnetic resonance ima­ging with magnetic resonance venography, be­cause of its non-invasiveness and high sensiti­vity, is the preferred modality for diagnosis and follow-up of cerebral venous thrombosis. [4]

Our patient made complete neurological reco­very. Early recognition, immediate institution of anticoagulation therapy and control of the nephrotic syndrome are essential measures to ensure a good prognosis. In conclusion, we wish to highlight the importance of evaluation for intra cranial disease when a nephrotic patient presents with persistent headache.

 Acknowledgement

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