,urnalof the Neurological Sciences, 111 (1992) 227-228 ,1992 Elsevier Science Publishers B.V. All rights reserved 0022-510X/92/$05.00
227
NS 03834
L e t t e r to t h e E d i t o r
Intracerebral haemorrhage in Sneddon's syndrome B . M . J . U i t d e h a a g a, p. S c h e l t e n s a, F . W . B e r t e l s m a n n a a n d R . P . M . B r u y n b a Department of Neurology, Free University Hospital, Amsterdam, The Netherlands, and b Department of Neurology, OudeurijnHospital Utrecht, Utrecht, The Netherlands (Received 23 March, 1992) (Accepted 6 April, 1992)
Sir, Sneddon's syndrome (Sneddon 1965) should be considered in all patients with generalized livedo racemosa and unexplained cerebrovascular events, especially in young women (Bruyn 1989). The most commonly found abnormalities in the cerebral arteries consist of (partial) occlusion of middle-sized arteries and irregularities of the vessel wall (Rebollo et al. 1983), resulting in cerebral infarction, often muitifocal with transient or longlasting neurological deficit. In 1987 we described a patient with Sneddon's syndrome in this journal (Bruyn et al. 1987). She was 37 years at the time of diagnosis. One year later epileptic seizures occurred for which she was treated with anticonvulsants. After a seizurefree period of 2 years the medication was discontinued. Three years later she was admitted to a local hospital because of a generalized seizure. Because she failed to fl:cover she was trattsferred to our hospital that same day. On admission ~,er Glasgow coma score was E1 M3 V2. There was flexion to pain on the right side and extension to pai:a on the left side. Cranial CT scan revealed a large Jntracerebral haemorrhage in the right hemisphere with e~ensio~ into the ventricular system (Fig. 1). Several angiographies in the past had never revealed an arteriovenous malformation (AVM), therefore it was decided to skip this procedure and to perform immediate neurosurgical intervention. The haemorrhage was evacuated and a ventricular catheter was inserted for external drainage. Unfortunately she never regained functional consciousness. She died 14 months after the operation in a nursing home at the age of 42. No autopsy was performed.
CorreslTondenceto:'Dr. B.M.J. Uitdehaag, Department of Neurology, Free University Hospital, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands.
The most common clinical neurological feature in Sneddon's syndrome is ischaemic cerebral infarction, however this patient illustrates the rare possibility of an intracranial haemorrhage. This might be caused by changes of the vessel wall. Because AVM's have been described in this syndrome (Rebollo et al. 1983) one c o u l d expect this to happen more often. However we found only one short de~ription of a patient who presented with cerebral haemorrhages in whom Sneddon's syndrome was diagnosed subsequently (DiezTejedor 1990). To our knowledge our patient is the first with the classical clinical picture of Sneddon's syndrome and the occurrence of an intracerebral haemorrhage in a later stage of the disease.
Fig. 1. CT scan showing the right-sided : With extension into the lateral ventricle. On the left side two wedge-shaped hypodense lesions illustrate the infarctions originating in the past.
228 References Bruyn, R.P.M. (1989) Sneddon's syndrome. In: Vinken, P.J., G.W. Bruyn and H.L. Klawans (Eds.), Handbook of Clinical Neurology, Vol. 11 (55), Elsevier Science Publishers, Amsterdam, pp. 401410. Bruyn, R.P.M., J.P.W. van tier Veen, A.J.M. Donker, J. Valk and E.C. Wolters (1987) Sneddon's syndrome. Case report and literature review. J. Neurol. Sci., 79: 243-253.
Diez-Tejedor, E., M. Lara, A. Frank, M. Guti6rrez and P. Barreiro (1990) Cerebral haemorrhage in Sneddon's syndrome. J. Neurol., 237 (Suppl.): $78. Rebollo, M., J.E Val, F. Carijo, F. Quintana and J. Berciano (1983) Livedo reticularis and cerebrovascular lesions (Sneddon's syndrome) ~ Clinical, radiological and pathological features in eight cases. Brain, 106: 965-979. Sneddon, I.B. (1965) Cerebrovascalar lesions and livedo reticularis. Br. $. DermaloL, 77: 180-185.
227
NS 03834
L e t t e r to t h e E d i t o r
Intracerebral haemorrhage in Sneddon's syndrome B . M . J . U i t d e h a a g a, p. S c h e l t e n s a, F . W . B e r t e l s m a n n a a n d R . P . M . B r u y n b a Department of Neurology, Free University Hospital, Amsterdam, The Netherlands, and b Department of Neurology, OudeurijnHospital Utrecht, Utrecht, The Netherlands (Received 23 March, 1992) (Accepted 6 April, 1992)
Sir, Sneddon's syndrome (Sneddon 1965) should be considered in all patients with generalized livedo racemosa and unexplained cerebrovascular events, especially in young women (Bruyn 1989). The most commonly found abnormalities in the cerebral arteries consist of (partial) occlusion of middle-sized arteries and irregularities of the vessel wall (Rebollo et al. 1983), resulting in cerebral infarction, often muitifocal with transient or longlasting neurological deficit. In 1987 we described a patient with Sneddon's syndrome in this journal (Bruyn et al. 1987). She was 37 years at the time of diagnosis. One year later epileptic seizures occurred for which she was treated with anticonvulsants. After a seizurefree period of 2 years the medication was discontinued. Three years later she was admitted to a local hospital because of a generalized seizure. Because she failed to fl:cover she was trattsferred to our hospital that same day. On admission ~,er Glasgow coma score was E1 M3 V2. There was flexion to pain on the right side and extension to pai:a on the left side. Cranial CT scan revealed a large Jntracerebral haemorrhage in the right hemisphere with e~ensio~ into the ventricular system (Fig. 1). Several angiographies in the past had never revealed an arteriovenous malformation (AVM), therefore it was decided to skip this procedure and to perform immediate neurosurgical intervention. The haemorrhage was evacuated and a ventricular catheter was inserted for external drainage. Unfortunately she never regained functional consciousness. She died 14 months after the operation in a nursing home at the age of 42. No autopsy was performed.
CorreslTondenceto:'Dr. B.M.J. Uitdehaag, Department of Neurology, Free University Hospital, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands.
The most common clinical neurological feature in Sneddon's syndrome is ischaemic cerebral infarction, however this patient illustrates the rare possibility of an intracranial haemorrhage. This might be caused by changes of the vessel wall. Because AVM's have been described in this syndrome (Rebollo et al. 1983) one c o u l d expect this to happen more often. However we found only one short de~ription of a patient who presented with cerebral haemorrhages in whom Sneddon's syndrome was diagnosed subsequently (DiezTejedor 1990). To our knowledge our patient is the first with the classical clinical picture of Sneddon's syndrome and the occurrence of an intracerebral haemorrhage in a later stage of the disease.
Fig. 1. CT scan showing the right-sided : With extension into the lateral ventricle. On the left side two wedge-shaped hypodense lesions illustrate the infarctions originating in the past.
228 References Bruyn, R.P.M. (1989) Sneddon's syndrome. In: Vinken, P.J., G.W. Bruyn and H.L. Klawans (Eds.), Handbook of Clinical Neurology, Vol. 11 (55), Elsevier Science Publishers, Amsterdam, pp. 401410. Bruyn, R.P.M., J.P.W. van tier Veen, A.J.M. Donker, J. Valk and E.C. Wolters (1987) Sneddon's syndrome. Case report and literature review. J. Neurol. Sci., 79: 243-253.
Diez-Tejedor, E., M. Lara, A. Frank, M. Guti6rrez and P. Barreiro (1990) Cerebral haemorrhage in Sneddon's syndrome. J. Neurol., 237 (Suppl.): $78. Rebollo, M., J.E Val, F. Carijo, F. Quintana and J. Berciano (1983) Livedo reticularis and cerebrovascular lesions (Sneddon's syndrome) ~ Clinical, radiological and pathological features in eight cases. Brain, 106: 965-979. Sneddon, I.B. (1965) Cerebrovascalar lesions and livedo reticularis. Br. $. DermaloL, 77: 180-185.
