Spontaneous intracerebral haemorrhage in patients suspected of multiple sclerosis

Abstract

SUMMARY Two cases of spontaneous intracerebral haemorrhage in adolescent girls suspected of having multiple sclerosis are reported. Surgical evacuation of haematomas in the left thalamus and left side of the pons, respectively, was performed with excellent recovery in both cases. Some clinical and pathological aspects of small arteriovenous malformations are discussed and the pertinent literature reviewed.The purpose of this report is to describe two cases of spontaneous intracerebral haemorrhage, both in adolescent girls, in which the clinical diagnosis was initially thought to be multiple sclerosis. Early in the course, the cerebrospinal fluid (CSF) was normal and later cerebral arteriograms and air-studies revealed mass lesions in the thalamus and pons respectively. Both patients improved considerably after surgical evacuation of haematomas at these sites. Neither case had evidence of hypertension, blood disease, arteritis, neoplasm, or mycotic aneurysm. The cerebral arteriograms were negative for berry aneurysm and large arteriovenous malformation. No history of head trauma was obtained.In recent years, Margolis, Odom, Woodhall, and Bloor (1951) and Russell (1954) have stressed the role of small arteriovenous malformations (AVM) in the production ofspontaneous intracerebral haematomas in young adults and children. For several reasons (vide infra) we think that the intracerebral haematomas in our cases were due to bleeds from so-called 'cryptic' AVM (Crawford and Russell, 1956;McCormick and Nofzinger, 1966). The striking feature of both clinical presentations was the tendency for remissions and exacerbations which initially suggested multiple sclerosis as the diagnosis. natal development had been normal; she had had no past neurological illnesses. Her parents and four siblings were well.In April 1964, she developed mild occipital headache and the next day had weakness and a numb feeling in her right side. She also complained of numbness of her left cheek and gums, diplopia on looking towards the left, and some difficulty with hearing on that side. The persistence of the right-sided weakness during the ensuing week resulted in her admission to the Children's Hospital Medical Center, Boston.Initial examination revealed a slender girl with normal vital signs and systemic examination; no cranial bruit was heard. Her mental status was normal and no dysphasia noted. Examination of her optic fundi, visual fields, and pupillary reactions were normal. On looking towards the left, horizontal nystagmus and diplopia were present, yet ocular movements had a full range. The left corneal reflex was depressed and sensation to touch and pinprick was decreased over the left forehead and cheek. Weakness of the facial muscles on the right was noted. Mild leftsided nerve deafness was confirmed by audiometry; rapid auditory fatigue and an absence of loudness recruitment were also found. She tended to fall to the right on walking and had a poor arm swing on that side; ankle clonus, hyperreflexia, and an extensor plantar response we...

Vascular malformations of the thalamus with normal angiograms

“…Infratentorial vascular malformations that produce waxing and waning symptoms of the posterior fossa dysfunction can mimic multiple sclerosis (1,4), and the diagnosis of vascular malformation is made usually after a fatal hemorrhage (11). Less commonly, posterior fossa vascular malformations are mistaken for tumors.…”

Vascular malformations of the thalamus with normal angiograms

“…Infratentorial vascular malformations that produce waxing and waning symptoms of the posterior fossa dysfunction can mimic multiple sclerosis (1,4), and the diagnosis of vascular malformation is made usually after a fatal hemorrhage (11). Less commonly, posterior fossa vascular malformations are mistaken for tumors.…”

Successful removal of an intrapontine haematoma.

“…Intra-axial haematomas are not distinguishable from intrinsic brainstem expanding tumours, which are generally not considered suitable for surgical therapy. Gros et al (1948), Scoville and Poppen (1949), Kempe (1964), Matson (1968), Dandy (1969), Obrador et al (1970) Abroms et al (1971) Kowada et al (1971 and Papo et al (1976) reported successful evacuation of subependymal brainstem haematomas. Myers et al (1961) Kowada et al (1971, and Scott et al (1973) reported successful evacuation of intraaxial brainstem haematoma.…”

“…Kempe (1964) described a brainstem haematoma presenting with Wallenberg's syndrome. Abroms et al (1971) described a case in which multiple sclerosis was established at the first stage and later successful evacuation of a pontine haematoma was performed. Spontaneous haemorrhage into the pons in children and adolescents is usually considered to be caused by a vascular malformation.…”

Surgical treatment of cryptic AVM's and associated hematoma in the brain stem and spinal cord

“…chiatry 19:[1][2][3][4][5][6][7][8][9][10][11] 1956 14.Dandy WE: The Brain. New York: Harper & Row, 1969, pp 642-643 15.…”

Vascular malformations of the thalamus with normal angiograms

“…Infratentorial vascular malformations that produce waxing and waning symptoms of the posterior fossa dysfunction can mimic multiple sclerosis (1,4), and the diagnosis of vascular malformation is made usually after a fatal hemorrhage (11). Less commonly, posterior fossa vascular malformations are mistaken for tumors.…”

Successful removal of an intrapontine haematoma.

“…Intra-axial haematomas are not distinguishable from intrinsic brainstem expanding tumours, which are generally not considered suitable for surgical therapy. Gros et al (1948), Scoville and Poppen (1949), Kempe (1964), Matson (1968), Dandy (1969), Obrador et al (1970) Abroms et al (1971) Kowada et al (1971 and Papo et al (1976) reported successful evacuation of subependymal brainstem haematomas. Myers et al (1961) Kowada et al (1971, and Scott et al (1973) reported successful evacuation of intraaxial brainstem haematoma.…”

“…Kempe (1964) described a brainstem haematoma presenting with Wallenberg's syndrome. Abroms et al (1971) described a case in which multiple sclerosis was established at the first stage and later successful evacuation of a pontine haematoma was performed. Spontaneous haemorrhage into the pons in children and adolescents is usually considered to be caused by a vascular malformation.…”

Surgical treatment of cryptic AVM's and associated hematoma in the brain stem and spinal cord

“…chiatry 19:[1][2][3][4][5][6][7][8][9][10][11] 1956 14.Dandy WE: The Brain. New York: Harper & Row, 1969, pp 642-643 15.…”

Vascular malformations of the thalamus with normal angiograms

“…Infratentorial vascular malformations that produce waxing and waning symptoms of the posterior fossa dysfunction can mimic multiple sclerosis (1,4), and the diagnosis of vascular malformation is made usually after a fatal hemorrhage (11). Less commonly, posterior fossa vascular malformations are mistaken for tumors.…”

Successful removal of an intrapontine haematoma.

“…Intra-axial haematomas are not distinguishable from intrinsic brainstem expanding tumours, which are generally not considered suitable for surgical therapy. Gros et al (1948), Scoville and Poppen (1949), Kempe (1964), Matson (1968), Dandy (1969), Obrador et al (1970) Abroms et al (1971) Kowada et al (1971 and Papo et al (1976) reported successful evacuation of subependymal brainstem haematomas. Myers et al (1961) Kowada et al (1971, and Scott et al (1973) reported successful evacuation of intraaxial brainstem haematoma.…”

“…Kempe (1964) described a brainstem haematoma presenting with Wallenberg's syndrome. Abroms et al (1971) described a case in which multiple sclerosis was established at the first stage and later successful evacuation of a pontine haematoma was performed. Spontaneous haemorrhage into the pons in children and adolescents is usually considered to be caused by a vascular malformation.…”

Surgical treatment of cryptic AVM's and associated hematoma in the brain stem and spinal cord

“…chiatry 19:[1][2][3][4][5][6][7][8][9][10][11] 1956 14.Dandy WE: The Brain. New York: Harper & Row, 1969, pp 642-643 15.…”