Venous Angloma of the Opti~Chiasm Joseph Fermaglich, MD, Jorge Kattah, MD, and Herbert Manz. MD
A 30-year-old man presented with decreased vision in the right eye of three weeks' duration. Examination indicated a chiasmal syndrome and evidence of subarachnoid hemorrhage. C A T scan showed a large suprasellar mass. Surgical intervention confirmed the presence of a globular lesion filled with blood clots involving mainly the right side of the optic chiasm. Following removal of the clots, the chiasm regained its shape and anatomical landmarks. Biopsy of the mass demonstrated a venous angioma located within the optic chiasm as well as recent and old bleeding. Following surgery the patient did well and recovered vision in the right eye, though a left homonymous hemianopia persisted.
Fermaglich J, Kattah J, Manz H: Venous angioma of the optic chiasm. Ann Neurol 4:470-471, 1978
Angiomas of the central nervous system are highly variable in size, location, and histological appearance [4]. I n contrast to large arteriovenous malformations of supratentorial distribution, small cryptic angiomas seldom affect t h e visual pathways [I]. This paper describes the clinical, anatomical, and pathological characteristics of an intrachiasmal venous angioma complicated by hematoma and subarachnoid bleeding. A 30-year-old man offered a three-week history of progressive visual loss in thc right eye associatcsd with intermittent pulsating bifrontal headaches, nausea, and vomiting. The patient was in no distress and vital functions were normal. Visual acuity in the left eye was 20/20, but in the right eye only hand motion was perceived at 60 cm. Hc failed to recognize the lshihara color plates with the right eye, yet read them correctly with the left eye. Visual field examination using a Goldman perimeter demonstrated a large relative central scotoma and enlargement of the blind spot in the right eye; the left visual field showed two scotomas: one was a relative central scotoma (IV,isopter), and thc second was a relative paracentral scotoma (IV4 isopter). A relative temporal hemianopia, sparing one small superoremporal area of vision, was found in the left visual field (Figurc). The patient was unable to identify red color From the Deparunent of Ncurology, Division of Neuroophthalmology and Division of Neuropathology, Georgetown Univcrsity Medical Ccntcr, Washington, DC.
Accepted for publication Mar 28, 1078.
Address reprint requests to Dr Fermaglich, 3800 Reservoir Rd, NW. Washington, DC 20007.
in either temporal field. The swinging flashlight test showed a Marcus Gunn pupil on the right; the pupils wcrc' otherwise normal. Funduscopic examination showed discrete bitemporal pallor. The remainder of t h e posterior pole and retina was normal. Extraocular motility was normal. With the exception of mild nuchal rigidity, the neurological examination showed no abnormalities. Skull roentgenograms and polytomograms of the sclla turcica and optic canals were normal. An enhanced CAT scan revealed a large suprasellar m a s occupying the suprasellar cistern. Carotid and vertcsbral arteriograms showed no abnormalities. Neuroendocrine evaluation was within normal limits. Cerebrospinal fluid was under an opening pressure of 14.0 cm H 2 0 . The HuiJ was xanthochromic and contained 786 red blood cclls and 31 white cells, all lymphocytes. Protein was 55, mg per deciliter and glucose, 56 mg p e r deciliter (blood glucose, 120 mgldl). Bacterial and fungal cultures were negative, as were acid-fast bacilli and cryptococcal antigen responses. Serological test for syphilis was nonreactive, and cytological examination showed no evidence of malignancy. Through a right frontal craniotomy a large, globular mass was identified in the right optic nerve just proximal to the optic foramen, extending medially and posteriorly to involve the left optic nerve and right optic tract. The lesion contained patches of bluish discoloration and numerous blue cysts. Necrotic tumor and clots were removed, and a biopsy of t h e mass was obtained. T h e postoperative course was uneventful. Neuroophthalmological examination showcd improvement of central vision in the right eye to 20/65+ with the capability of reading 3 of 12 Ishihara color plates. Acuity in thc left eye remained 20/20+. Visual ficld examination revealed a dense left homonymous hemianopia in addition to a relative central scotoma in the left eye. The rest of the neuroophthalmological and general neurological examinations remained normal. The striking pathological feature was the presence of numerous irregular vascular channels in the optic nerve. An internal elastic membrane was not identified in thc abnormal vessels, s o venous angioma appeared to be the likely diagnosis.
Discussion Small, asymptomatic vascular malformations are not infrequently found at autopsy [ 3,5] and usually are of three histological types: capillary telangiectasias, vcnous angiomas, and artcriovcnous malformations. Most commonly they are infratentorial and, although clinically silent, rarely may be the source of otherwise unexplained subarachnoid bleeding [ 51. Primary vascular abnormalities involving the optic chiasm exclusively must be exceedingly rare since only 2 cases have been reported in which a cryptic angioma was suspected. Burnbaum noted t h e occurrence of a blue-domed cyst in the right optic nerve (personal communication, 1977). He postulated that o n e small arteriovenous malformation was the origin
470 0364-5134/78/0004-0514501.25 @ 1978 by Joseph Fermaglich
Visualfield exarninution. A rdatiw ientralscotoma tuasfiund in the right eye and two relative scotomas were plotted i n the kft eye. Thesefindings mggest bilateral optic news invulcemsnt.
of an intraneural blood clot. Following hemorrhage, small vascular defects are usually destroyed and not dernonstrablc [ 5 ] . Dandy [2] reported a patient with chronic chiasmal syndrome secondary to compression by a large mass of vcins situated anterior to the chiasm between the two optic nerves. No pathological examination was available. Finally, Walsh and Hoyt [b]found optic tract involvement in 3 instances of basally situated arteriovenous malformations. Prcliminary histological examination was performed by Larry Klcin, MD, and Byungkyu Chun, MD. T h e authors cxpress their gratitude to Margic Lyons for cdirorial assisrance.
References 1. Bynke H, Ihoksson P: Visual field defects in arteriovenous
aneurysms of the brain. Acta Ophthalmol 36586-599, 1958 2. Dandy WE. Venous abnormalities and angiomas of rhe brain. Arch Surg 1 7:7 15-793, 1‘928 3. McCormick WF. The pathology of vascular (arreriovenous) malformations. J Neurosurg 24807-8 16. 1966 4. McCormick WF, Hardman JM, Boultcr RT: Vaccular malfurmations (angiomas) of the brain. with special reference to those occurring in thc posterior fossa. J Neurosurg 28:24 1-2 5 1,
I968 5. McCormic k WF, Nofsingcr JD: Cryptic vascular malformation of tht. CNS. J Neurosurg 24:865-875, 1966 6. Wdsh FB. Hoyt WT: Clinical Neuro-ophthalmology. Balrimore. Williams & Wilkins. 1960. VOI 2, pp 1708-1709
Downbeat N ystagmus due to Anticonvulsant Toxicity Jack N. Alpcrt, MD
Isolated downbeat nystagmus was observed in 2 patients on multiple anticonvulsant regimens. The nystagmus disappeared when phenytoin dosage was reduced. Electrooculographic analysis revealed impaired downward tracking, supporting the concept of “pursuit” nystagmus. Alpert JN: Downbeat n y s t a p u s due to anticonvulsant toxicity. Ann Neurol4:471-473, 1978
Downbeat nystagmus is an unusual neurological sign usually atrributed to a lesion affecting the caudal medulla. There are no previous reports of drug intoxication producing downbeat nystagmus. T w o cascs of downbeat nystagmus secondary to anticonvulsant intoxication are reported. Patient I A 19-year-old girl had had a history of poorly controlled seizurcs since childhood, rnanifestcd by grinning, staring, ~~~
From the Neurology Department, Sr. Luke’s Episcopal Hospital. Houston, TX. Accepted for publication Apr 24, 1978. Address reprint requests to D r Alpert, Suirc 910, Hermann Profcssional Bldg, Houston. T X 77030.
0364-5134/78/0004-0515$01.25 @ 1978 by Jack N. Alpert
471
A 30-year-old man presented with decreased vision in the right eye of three weeks' duration. Examination indicated a chiasmal syndrome and evidence of subarachnoid hemorrhage. C A T scan showed a large suprasellar mass. Surgical intervention confirmed the presence of a globular lesion filled with blood clots involving mainly the right side of the optic chiasm. Following removal of the clots, the chiasm regained its shape and anatomical landmarks. Biopsy of the mass demonstrated a venous angioma located within the optic chiasm as well as recent and old bleeding. Following surgery the patient did well and recovered vision in the right eye, though a left homonymous hemianopia persisted.
Fermaglich J, Kattah J, Manz H: Venous angioma of the optic chiasm. Ann Neurol 4:470-471, 1978
Angiomas of the central nervous system are highly variable in size, location, and histological appearance [4]. I n contrast to large arteriovenous malformations of supratentorial distribution, small cryptic angiomas seldom affect t h e visual pathways [I]. This paper describes the clinical, anatomical, and pathological characteristics of an intrachiasmal venous angioma complicated by hematoma and subarachnoid bleeding. A 30-year-old man offered a three-week history of progressive visual loss in thc right eye associatcsd with intermittent pulsating bifrontal headaches, nausea, and vomiting. The patient was in no distress and vital functions were normal. Visual acuity in the left eye was 20/20, but in the right eye only hand motion was perceived at 60 cm. Hc failed to recognize the lshihara color plates with the right eye, yet read them correctly with the left eye. Visual field examination using a Goldman perimeter demonstrated a large relative central scotoma and enlargement of the blind spot in the right eye; the left visual field showed two scotomas: one was a relative central scotoma (IV,isopter), and thc second was a relative paracentral scotoma (IV4 isopter). A relative temporal hemianopia, sparing one small superoremporal area of vision, was found in the left visual field (Figurc). The patient was unable to identify red color From the Deparunent of Ncurology, Division of Neuroophthalmology and Division of Neuropathology, Georgetown Univcrsity Medical Ccntcr, Washington, DC.
Accepted for publication Mar 28, 1078.
Address reprint requests to Dr Fermaglich, 3800 Reservoir Rd, NW. Washington, DC 20007.
in either temporal field. The swinging flashlight test showed a Marcus Gunn pupil on the right; the pupils wcrc' otherwise normal. Funduscopic examination showed discrete bitemporal pallor. The remainder of t h e posterior pole and retina was normal. Extraocular motility was normal. With the exception of mild nuchal rigidity, the neurological examination showed no abnormalities. Skull roentgenograms and polytomograms of the sclla turcica and optic canals were normal. An enhanced CAT scan revealed a large suprasellar m a s occupying the suprasellar cistern. Carotid and vertcsbral arteriograms showed no abnormalities. Neuroendocrine evaluation was within normal limits. Cerebrospinal fluid was under an opening pressure of 14.0 cm H 2 0 . The HuiJ was xanthochromic and contained 786 red blood cclls and 31 white cells, all lymphocytes. Protein was 55, mg per deciliter and glucose, 56 mg p e r deciliter (blood glucose, 120 mgldl). Bacterial and fungal cultures were negative, as were acid-fast bacilli and cryptococcal antigen responses. Serological test for syphilis was nonreactive, and cytological examination showed no evidence of malignancy. Through a right frontal craniotomy a large, globular mass was identified in the right optic nerve just proximal to the optic foramen, extending medially and posteriorly to involve the left optic nerve and right optic tract. The lesion contained patches of bluish discoloration and numerous blue cysts. Necrotic tumor and clots were removed, and a biopsy of t h e mass was obtained. T h e postoperative course was uneventful. Neuroophthalmological examination showcd improvement of central vision in the right eye to 20/65+ with the capability of reading 3 of 12 Ishihara color plates. Acuity in thc left eye remained 20/20+. Visual ficld examination revealed a dense left homonymous hemianopia in addition to a relative central scotoma in the left eye. The rest of the neuroophthalmological and general neurological examinations remained normal. The striking pathological feature was the presence of numerous irregular vascular channels in the optic nerve. An internal elastic membrane was not identified in thc abnormal vessels, s o venous angioma appeared to be the likely diagnosis.
Discussion Small, asymptomatic vascular malformations are not infrequently found at autopsy [ 3,5] and usually are of three histological types: capillary telangiectasias, vcnous angiomas, and artcriovcnous malformations. Most commonly they are infratentorial and, although clinically silent, rarely may be the source of otherwise unexplained subarachnoid bleeding [ 51. Primary vascular abnormalities involving the optic chiasm exclusively must be exceedingly rare since only 2 cases have been reported in which a cryptic angioma was suspected. Burnbaum noted t h e occurrence of a blue-domed cyst in the right optic nerve (personal communication, 1977). He postulated that o n e small arteriovenous malformation was the origin
470 0364-5134/78/0004-0514501.25 @ 1978 by Joseph Fermaglich
Visualfield exarninution. A rdatiw ientralscotoma tuasfiund in the right eye and two relative scotomas were plotted i n the kft eye. Thesefindings mggest bilateral optic news invulcemsnt.
of an intraneural blood clot. Following hemorrhage, small vascular defects are usually destroyed and not dernonstrablc [ 5 ] . Dandy [2] reported a patient with chronic chiasmal syndrome secondary to compression by a large mass of vcins situated anterior to the chiasm between the two optic nerves. No pathological examination was available. Finally, Walsh and Hoyt [b]found optic tract involvement in 3 instances of basally situated arteriovenous malformations. Prcliminary histological examination was performed by Larry Klcin, MD, and Byungkyu Chun, MD. T h e authors cxpress their gratitude to Margic Lyons for cdirorial assisrance.
References 1. Bynke H, Ihoksson P: Visual field defects in arteriovenous
aneurysms of the brain. Acta Ophthalmol 36586-599, 1958 2. Dandy WE. Venous abnormalities and angiomas of rhe brain. Arch Surg 1 7:7 15-793, 1‘928 3. McCormick WF. The pathology of vascular (arreriovenous) malformations. J Neurosurg 24807-8 16. 1966 4. McCormick WF, Hardman JM, Boultcr RT: Vaccular malfurmations (angiomas) of the brain. with special reference to those occurring in thc posterior fossa. J Neurosurg 28:24 1-2 5 1,
I968 5. McCormic k WF, Nofsingcr JD: Cryptic vascular malformation of tht. CNS. J Neurosurg 24:865-875, 1966 6. Wdsh FB. Hoyt WT: Clinical Neuro-ophthalmology. Balrimore. Williams & Wilkins. 1960. VOI 2, pp 1708-1709
Downbeat N ystagmus due to Anticonvulsant Toxicity Jack N. Alpcrt, MD
Isolated downbeat nystagmus was observed in 2 patients on multiple anticonvulsant regimens. The nystagmus disappeared when phenytoin dosage was reduced. Electrooculographic analysis revealed impaired downward tracking, supporting the concept of “pursuit” nystagmus. Alpert JN: Downbeat n y s t a p u s due to anticonvulsant toxicity. Ann Neurol4:471-473, 1978
Downbeat nystagmus is an unusual neurological sign usually atrributed to a lesion affecting the caudal medulla. There are no previous reports of drug intoxication producing downbeat nystagmus. T w o cascs of downbeat nystagmus secondary to anticonvulsant intoxication are reported. Patient I A 19-year-old girl had had a history of poorly controlled seizurcs since childhood, rnanifestcd by grinning, staring, ~~~
From the Neurology Department, Sr. Luke’s Episcopal Hospital. Houston, TX. Accepted for publication Apr 24, 1978. Address reprint requests to D r Alpert, Suirc 910, Hermann Profcssional Bldg, Houston. T X 77030.
0364-5134/78/0004-0515$01.25 @ 1978 by Jack N. Alpert
471
