Clinical Ncwrology und Neurosurgery, 94 (1992)

0 1992 Elsevier Science Publishers



II-I 3

B.V. All rights reserved




Cranial nerve involvement in childhood polyarteritis nodosa Rezan Topaloglu,

Nesrin Besbas, Umit Saatci, Aysin Bakkaloglu

(Received (Revised,


Kry ir,ords:




7 January.

received 2X March


and Ayse Oner



and IO July, 1991)

16 July. 1991)

nerve involvement


Amongst unusual.

a variety

of neurological


We report 4 cases with cranial

of childhood



nerve palsies in a series of 36 biopsy-proven



nerve involvement

Two cases presented

IIIrd nerve palsy alone, one with right IIIrd and left IVth nerve palsy. and one with peripheral VIIth nerve paresis. 4 patients showed good response to prednisolone and cyclophosphamide treatment. Cranial nerve involvement childhood polyarteritis nodosa seems not so rare when patients are followed on long term basis.

All in

sedimentation rate (ESR) at I 18 mm/h, other laboratory findings were normal. A skin and muscle biopsy showed



is Mith


(PAN) is characterized

by a focal


and medium-sized


PAN was diagnosed

necrotizing inflammation of small and medium-sized arteries. A great variety of clinical features results from the diverse distribution of these arterial lesions [l-3].

and prednisolone (60 mgim’iday) was prescribed. She was lost to control for 3 years. when she presented again with rashes and myalgia. A second course of predniso-

Nervous system involvement in PAN is reported to range between 14 and 72% in different series 14-91. Despite a high frequency of neurological manifestations.

lone was initiated. While on prednisolone 20 mgiday, she developed unsteadiness. nausea and double vision. On neurological examination, her visual acuity. fundoscopy


and pupil reactions


nerve palsies are rare [lO,l 11. A total of

were normal.

She had left elevation

36 histopathologically proven PAN cases, aged between 4 and 12 years. were seen at our Department between

paresis and ptosis. Laboratory tests showed: ESR 46 mm/h. C3 70 mgidl (normal 60 IX), C4 3X mgidl ( 20

1979 and 1990. Among these, 4 cases had cranial nerve involvement. We report here our findings of these specific cases.

40). ANA (-). anti-DNA 4 pgidl (O-25). An axial CT scan of the head was normal. She was started on cyclophosphamide 2.5 mglkglday. The dosage of prednisolone was kept unchanged. Within 1 week her symptoms improved.

Case reports








fever of long duration, rash on the extremities, abdominal pain and arthralgia. Except an elevated erythrocyte

Corr~.~/Jont~~nc,[, IO: Dr. Rezan Nephrology.



Topaloglu, Hospital,

Department 06100 Ankara,

of Pediatric Turkey.

A h-year-old boy. the brother of case No. 3. presented with fever of unknown origin. abdominal pain and rash on extremities.




ESR (50 mm/h). C-reactive protein (CRP) was (++). latex fixation test (+). A skin and muscle biopsy compatible with PAN. Initial treatment trials failed to poor parental compliance. Eighteen months later.

high and was due


patient was admitted to hospital with the complaint of’ double vision. His visual acuity. fundoscopy and pupil reactions were normal. He had right IIIrd and left IVth nerve palsies. He was started on prednisolone (60 mglm’i day) and cyclophosphamide (2.5 mglkglday). The patient recovered gradually within a period of 3 months. Case No. 3

This 1l-year-old boy is the brother of case No. 2. He presented with chronic fever, pain in the extremities and arthralgia of the small joints. He had hypertension and minimal pericardial effusion. His ESR was elevated (60 mm/h) and CRP was (+). A skin and muscle biopsy showed small and medium-sized arteritis. Three years later, while on low-dose prednisolone ( 10 mgikg), he developed complaints of hypoesthesia in median nerve distribution and weakness in dorsiflexion of the right hand. One year later he presented with double vision and ptosis. On neurological examination his visual acuity and fundoscopy were normal. There was a left total (mydriasis and diminished light reaction), IIIrd cranial nerve palsy with complete ptosis. An axial CT scan of the head was normal. He was put on cyclophosphamide (2.5 mg/ kg) in addition to prednisolone (10 mg/day). Six months later the ocular movements had reverted to normal. Case No. 4

A 4.5-year-old boy presented with edema, rashes on the hands and feet, and pain in the extremities. Laboratory tests revealed a high ESR (55 mm/h) and CRP (++). A skin and muscle biopsy was compatible with PAN. Two months after initiation of prednisolone treatment he developed numbness and weakness of the left hand. Bolus methylprednisone therapy (30 mg/kg/day, maximum 1 g) was given in monthly intervals in addition to 10 mg/day prednisolone. His symptoms improved within 4 months. Two years later while he was on low-dose (10 mg/day) prednisolone he presented with a left-sided peripheral VIIth cranial nerve palsy. Cyclophophamide (2.5 mgikglday) was started in addition to prednisolone. In a period of 2 months his symptoms resolved.


Various neurological manifestations of PAN are caused by the small and middle-sized arterial vasculitis. Stages of inflammation, vessel wall infiltrates, necrosis, thrombosis, fibrosis, occlusion and scarring result in functional and structural alterations in peripheral (PNS)

or central (CNS) nervous system [ I .;j 1he inciciotu oi manifestations is 60% and 40%. re.spcctlvel~ 1I .I!. i 21. Cranial nerve palsies were rarely rcportcd in dwelt JMtients. In a series of 114 patients by Ford and Siekert /2] CNS complications were present in 36%. cranial ncrvc palsies in 8%. and ocular nerve involvement 111J’+. Moore and Cupps [3] reported the frequency of neurological manifestations of PAN as 30 co 40% of cases and cranial nerve findings have been reported to be less than frequent. In Moore and Fauci’s report [l]. which included 2 children, no cranial nerve involvement has been mentioned. We treated our patients with a combination of cyclophosphamide and prednisolone and neurological symptoms improved either directly or shortly after initiation of this therapy as has been suggested by others [ 13,141. We had the opportunity to follow-up our patients for at least 3 years each. Two of our patients presented with IIlrd nerve palsy, one with IIIrd and IVth nerve palsies on the right and left respectively. and one with peripheral unilateral facial palsy. In our series of 36 cases, 4 cases had neurological findings confined solely to cranial nerves. Ocular nerves were selectively diseased in 3. One of our cases showed a IIIrd nerve palsy with pupillary involvement as has been reported by Both [ 151in vasculitis. PAN is a rare disease in childhood. We were unable to find a series in which cases of PAN were studied to determine the occurrence and frequency of cranial nerve involvement in the childhood form of this particular disease. However, there is at least one sporadic case report [16]. The involvement of cranial nerves in 4 of 36 cases constitutes a higher percentage than that of the adult series published. Our figures suggest that the cranial nerve findings, aside from CNS or PNS complications, may be encountered more frequently than expected on the basis of literature data (contrary to classical reviews) during the long and protracted course of the disease. Children with PAN should be thoroughly examined at frequent intervals for any manifestation of cranial nerve palsy.


1 Moore, P.M. and Fauci, A.S. (1981) Neurologic manifestations of systemic vasculitis. Am. J. Med., 7 1: 517-524. 2 Ford, R.G. and Siekert, R.G. (1965) Central nervous system manifestations of periarteritis nodosa. Neurology, 15: 114-122.

13 3 Moore, cations

P.M. and Cupps, of vasculitis.

T.R. (1983) Neurological

Ann. Neurol..


4 Savage, C.O.S.. Winearls, C.G., Evans, D.J., Rees, A.J. and Lockwood, J.M. (1985) Microscopic polyarteritis: presentation. pathology and prognosis. Q.J. Med.. 56: 467483. 5 Serra. A.. Cameron, J.S. and Turner. D.R. et al. (1984) Vasculitis effecting the kidney: Presentation, histopathology and long-term outcome. Q. J. Med.. 53: 181-208. 6 Scott, D.G.I., Bacon, P.A., Eliott, P.J., Tribe, C.R. and Wallington, T.B. (1982) Systemic vasculitis in a District General Hospital, 1972 80: Prognosis in 80 cases. Q. J. Med., 51: 292231 I. 7 Leib. ES.. Restivo. C. and Paulus,

H.E. (1979) Immuno-

suppressive and corticosteroid therapy dosa. Am. J. Med.. 67: 941-947.

of polyarteritis

10 Ashwall, tions

14: 155163.


X Frohnertt, P.P. and Sheps. S.G. (1967) Long term follow up study of periarteritis nodosa. Am. J. Med., 43: 8814. 9 Adu. D.. Howie, A.J.. Scott. D.G.I.. Bacon, P.A., McGonigle. R.J.S. and Michael J. Polyarteritis and the kidney. Q. J. Med., 62: 221~ 237.

S. and Schneider,

of vasculitic

K.F. (Ed.), Pediatric

S. (1989) Neurologic

disorders Neurology

of childhood. Principles


In: Swaiman, and Practice Vol.

I. CV Mosby Co., St. Louis, MO. pp. 634-637. 11 Walsh, F.B. and Hoyt, W.F. (1969) Clinical Neuro-Ophthalmology, Williams and Wilkins. Baltimore, pp. 117% 1182. 12 Loushin. L.L. and Kerrohan. J.W. (1948) Peripheral neurites in periarteritis nodosa. Arch. Int. Med., 82: 321337. 13 Fauci, A.S., Katz, P., Haynes.

B.F. and Wolff, SM.


Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N. Engl. J. Med., 301: 2355238. 14 Fauci, A.S. (1978) The spectrum of v,ascuhtis. Clinical pathological, immunologic and therapeutic considerations. Ann. Intern. Med.. 89: 660- 676. I5 B&h, J. (1956) Ocular changes in periarteritis nodosa. Am. J. Ophthalmol.. 42: 567-575. 16 Case Records of the Massachusetts General Hospital (Case 43-1986). N. Engl. J. Med.. 315: 1143-l 154.


Cranial nerve involvement in childhood polyarteritis nodosa.

Amongst a variety of neurological manifestations of childhood polyarteritis nodosa, cranial nerve involvement is unusual. We report 4 cases with crani...
274KB Sizes 0 Downloads 0 Views