Cerebral infarction in juvenile rheumatoid arthritis A.K. Gururaj*, R. Pratap Chand” *, and S.P. Chuah* Case report Summary Z.M., a 2-year-old female child was first hospitalized in 1984 for fever and polyarthritis and was diagnosed to have seropositive polyarticular juvenile rheumatoid arthritis and was treated with aspirin. The response was good. She was subsequently admitted in February 1986 at the age of 5 years with a history of fever and fits of one day duration. The fits consisted of clonic movements of the left upper and lower limbs, progressing to involve the right upper and lower limbs associated with uprolling of the eye balls. There was no loss of consciousness. The episode lasted for a few seconds and was followed by post-ictal drowsiness. There was no past history of fits. She was a product of full term normal delivery born to non-consanguinous parents and there was no history of birth asphyxia. Her development had been normal. Examination revealed a fully conscious child who was febrile and pale. Her vital signs were normal. There were swellings and deformities in both wrists and knee joints. Utnar deviation of the hands at wrists was evident. Passive extension at the left knee was limited. No joint tenderness or contractures were found. Marked wasting of the muscles of the upper and lower limbs was observed. Neurologically, no deficits were elicitable. There was no hepatosplenomegaly or Iymphadenopathy. The kidneys were not pal- * Department of Paediatrics and * * Department Bharu, Malaysia Cerebral involvement associated with juvenile rheumatoid arthritis is rare. It is not influenced by treatment and the presentation can be varied. We describe a case of cerebra1 infarction secondary to vasculitis in a child with juvenile rheumatoid arthritis. Key words: Juvenile rheumatoid arthritis, cerebral vasculitis. pable. Cardiovascular and respiratory systems were normal. Following admission, the child continued to have fever and subsequently, her right ankle became painful. She had an episode of partial seizure involving the left upper and left lower limbs on the 3rd day. This was preceeded by an aura of formed visual hallucination in which she repeatedly saw her sister falling down from a bed. Following the second fit, she developed left sided hypotonic hyporeflexic hemiparesis and left upper motor neurone type of facial palsy. Barring the post-ictal drowsiness, there was no altered sensorium. There was no detectable papilledema. On the 4th day, she was noted to have mild jaundice and areas of cutaneous vasculitis were observed on the planter aspect of her great toes (Fig. 1). The results of the investigations are shown in Table 1. The latex ofNeurology,Hospital Vniversiti S&s Malaysia, Kubang Kerian, 16ISO Kota Address for correspondence and reprint requests: A. K. Gururaj, Department Kubang Kerian, 16150 Kota Bharu, KeIantan of Paediatrics, Hospital Universiti Sains Malaysia, Accepted 15.4.88 Clin Neural Neurosurg 1988. Vol. 90-3 261 Fig. 2. I’he Cl‘ scan with enhancement she\\mg ct~lL,br:ii infarction rheumatoid factor was positive 1:640 ad ESR was 90 mm/hour. The electroencephalogram (EEG) revealed focal polymorphic delta activity indicating an acute structural cortical lesion in the right parieto-occipital region and focal epilepsy originating from the right posterotemporal and occipital regions. CT scan showed a low density non-enhancing lesion in the right occipital region with pressure effects on the occipital horn of the right lateral ventricle and posterior falx cerebri (Fig. 2). The child continued to receive oral soluble aspirin (75 mg/kg/day). The fits were controlled with an intravenous Phenytoin infusion (7 mg/ kg) followed by oral Phenytoin (5 mg/kg/day). Her neurological recovery was rapid, the hemiparesis and facial palsy improved within three days. Presently she is asymptomatic. No residual hemiparesis or hemianopia have been observed. Discussion Cerebral involvement 262 associated with juvenile in the right occipital region rheumatoid arthritis (JRA) is rare but well documented. It may arise from cerebral vasculitis or rheumatoid nodules in the meninges or other intracranial structures’. In a review of 17Ocases, Jan et al. observed abnormal central nervous system functions in 13 (7.5 percent)‘. Dysfunction included drowsiness, seizures. irritability. incoordination, stupor, chorea, meningismus and papilledema. In all these cases, the spinal > -.-.I_- lnvestigatton Kehult Serum bilirubin fi9 urn&L Normal Values -__ E- I7 WnoVL Aspartate amino transferasc 101 IUIL s-15 IL’X. Alaninc amino transferasc 52 IU/L 4-12 IL/L Hepatitis A Virus 1gM HbSAg Cerebra spinal fluid Protein Glucose Chloride Glohuhn lmmunoglohulins IgG ‘gM IRA Blood tirca Sodium negative negative II-4 g/L 3.4 mmoVL 124 mmol/L negative 0. IS-O.6 g/L 2.1-3.75 mmoVL 120-130 mmoUL to.7 g/L 3.92 g/L 4.x5 e/r. 5-13 g/L 0.5-I .99 m&L 0.23-l .9 mg/L 1.0 mm&L 141 mmoYL 3.0-6.0 mm&L 135-145 mmoI/L fluid was essentially clear. dacksonian epilepsy resulting from focal intracranial lesion was first reported by Ellman et (11.in 1X4”, and is reported to be a less common manifestation. In the present report. the patient had complex partial seizures as evidenced by the aura and motor manifestations. The EEG described in JRA include persistent or intermittent diffuse slowing of the back~r~~und activity. or asymmetries of different degrees’l’,‘. The abnormal EEG findings are known to occur more often in girls than in boys and are associated with active disease. The EEG changes seen in this patient are similar to that described in the literature. The CT scan in this patient is suggestive of infarction possibly secondary to vasculitis. Vasculitis in JRA is characterized by fibrinoid necrosis of the media and inflammatory cell infiltration of the adventitia. Vasculitis can result in a variety of neurological syndromes mostly related to seyualae of vascular obliteration, thrombosis followed by infarcti(~n or rupture and haemorrhage’. The symptoms depend on the anatomical location of the lesion and the extent of the area involved. Rarely, the cerebral nlanifestations in JRA can be secondary to hypertensive encephalopathy associated with the use of steroids or hyperviscosity syndrome secondary to excessive serum proteins, such as cryoglobulinemia and cir- culating IgG Complexes. The neur(~logical progression may or may not be reversed by antiinflammatory drug therapy. In the present case, the cerebral infarction occurred when the patient was on Aspirin. Apart from Phenytoin, no other drugs were given and the child eventually made a full neurological recovery. In conclusion, we present a case of seropositive JRA with a focal neurological inv(~lvement due to cerebral infarction possibly secondary to vasculitis. References MAKELA hl.,LANG H.StI.LANPAAM. Neurolllgicaimanil’ehtations of rheumatoid arthritis. Ci W (Eds.) of clinical neurology stcrdam: Handbook North Holland In: Vinken Publishing PJ and Bruyn Part I. Am- Company. 1979; 38:470-99. JAN JE. Hfi.lRH, LOW juvenile rhcumatnid COmpiiG&XlS MD. CCr&rd arthritis. Can Med in Assoc .I !973: 107:623-5. ELLMAN JS. Widespread P. Cl_!DKOWITSL. ELRWOOD rous membrane se- inv~~l~ement by rheumat~~id nodules. .I Clin Path 19%; 7:239-M SILIANPAA M, LANG AH, KALIMO H. Natural juvenile rheumatoid arthritis. history of Acta paediatr Stand 197X: 67:537-41. LANGH, ANT-F1I.A R.SVEKL!S A, LA,XKSONEN Al..EEG ings in ,juvenile rheumatoid arthritis tive tissue diseases in children, find- and other connec- Acta Paediatr Stand 1974; 63:373-80. 263