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Linear Nevus Sebaceous Syndrome
Linear Nevus Sebaceous
Syndrome
H. MICHAEL LAMBERT, MD, JACK O. SIPPERLEY, MD, JOHN W. SHORE, MD, J. PAUL DIECKERT, MD, RICHARD EVANS, MD, DONALD K. LOWD, OT
Abstract: The linear nevus sebaceous syndrome is a rare disorder first described in 1962 in two cases by Feuerstein and Mims. As originally described, it consisted of the triad of the characteristic midline facial linear nevus sebaceous of Jadassohn, seizures, and mental retardation. The authors have followed a patient with this syndrome from birth to age 8 and although he does demonstrate several neurologic and ocular abnormalities, he enjoys normal intelligence and has never suffered seizures. The features of this syndrome and several new ocular findings demonstrated in this case are described. Based on our longterm follow-up of this patient and our review of the reported cases to date, the authors suggest that the triad for this disorder should be changed to include the midline facial linear nevus sebaceous of Jadassohn, neurologic abnormalities which may, but not necessarily, include seizures and mental retardation, and ophthalmologic abnormalities. [Key words: Feuerstein-Mims syndrome, linear nevus of Jadassohn, linear nevus sebaceum, phakomatosis, oculoneurocutaneous, osseous choristoma, subretinal neovascularization.] Ophthalmology 94:
278-282, 1987
In 1932, Van der Hoeve 1 first used the term "phakomatosis," meaning mother spot or birthmark, to describe the interrelationship of several congenital neurocutaneous syndromes including encephalotrigeminal angiomatosis (Sturge-Weber syndrome), neurofibromatosis (Von Recklinghausen's disease), tuberous sclerosis (Bourneville's disease), and angiomatosis of the retina and cerebellum (Von Hippel-Lindau disease). Ataxia-telangiectasia (Louis-Bar syndrome) was later added to this group. In 1963, Feuerstein and Mims2 described two patients with a new neurocutaneous disorder consisting of the triad of a characteristic midline facial skin lesion (linear sebaceous nevus of Jadassohn), seizures, and mental retardation. Since their original description, other cases of this syndrome with a growing list of associated lesions have been reported. We describe a case of the linear nevus seFrom the Department of Ophthalmology, Wilford Hall USAF Medical Center, San Antonio. The views expressed herein are those of the authors and do not necessarily reflect those of the US Air Force or the Department of Defense. Reprint requests to H. Michael Lambert, LtCoI, USAF, MC, Department of Ophthalmology (SGHSE), Wilford Hall USAF Medical Center, San Antonio, TX 78236.
278
baceous syndrome with 8 years of follow-up (previously described at the time of presentation3). Based on this case and a review of the literature, we suggest a modification of Feuerstein and Mims' original triad.
CASE REPORT In 1977, a 5-month-old white boy was referred to Wilford Hall USAF Medical Center for evaluation of an unusual facial lesion. He was the product of an uncomplicated term pregnancy and normal vaginal delivery. Family history was negative for any significant illnesses, skin lesions, seizures, or mental retardation. On physical examination, the patient was found to have a pale yellow plaque on the left side of his face extending from the scalp to the jaw and anteriorly to the midline. The lesion was smooth in appearance in the scalp area, becoming more verrucous in the area of the jaw. Marked alopecia was apparent in the left fronto-parietal region (Fig I). No other skin lesions were present. On neurologic examination, the patient was found to have normal psychomotor development with a head circumference at the 50th percentile. He had no history of seizures and had a
LAMBERT, et al • LINEAR NEVUS
Fig 1. Top leji and right. front and side views. The facial lesion extended from the scalp to the jaw and to the midline (age 5 months). The lesion was smooth in parietal region, becoming more verrucous in the area of the jaw. The left lid was notched and held in a narrowed position. There was marked alopecia of the fronto-parietal region. Fig 2. Bottom leji, CT shows mild atrophy of the left cerebral and cerebellar hemispheres and a large arachnoid cyst. Fig 3. Bottom right, conjunctival choristomatous lesions ofleft eye extending into cornea.
normal electroencephalogram (EEG). A computed tomography
(Cf) scan demonstrated moderate atrophy of the left cerebral
and cerebellar hemispheres and a large arachnoid cyst (Fig 2). On ophthalmologic examination, he was able to fix and follow with the right eye, but had poor fixation in the left. The left upper eyelid was notched, and the fissure was narrowed by the nevus sebaceum. The coloboma involved the puncta and canaliculus. Apparent lipodermoids were found in the superior and inferior conjunctiva of the left eye and in the superior cul-desac of the right (Fig 3). Ductions in the left eye were markedly
reduced with a left exotropia and hypotropia with the right eye fixating. Results offundus examination showed a disc coloboma in the left eye and bilateral "pale areas" superior to the disc in the right eye and surrounding the disc and extending into the temporal periphery in the left. Amblyopia therapy was instituted immediately but vision failed to improve in the left eye. Over the past 8 years, the patient has been followed jointly by plastic surgery, neurology, and ophthalmology. He has undergone serial excisions of his facial lesions, and hair-bearing tissue has been moved to the area of alopecia. The majority of
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the facial nevus has been removed. The patient has normal intelligence and has had no seizures. He does have a perplexing mild atrophy of the left extremities. Results of repeat fundus examinations have shown the "pale areas" to be yellow-white choroidal masses. In the right eye, the lesion now extends from the disc superiorly, and in the left eye the lesion surrounds the disc and extends temporally. cr scans of the orbits have confirmed the diagnosis of bilateral choroidal osteomas (Fig 4). At age 7, cosmetic repair of his left hypotropia and exotropia was attempted, but at surgery the superior rectus muscle was found to be absent. The remaining extraocular muscles were surrounded and infiltrated by fibro-fatty tissue, which was partially excised. Partial correction of his ptosis was attained by a silicone frontalis sling. The eyelid coloboma was closed primarily. At age 8, amacular subretinal neovascular membrane developed over the choroidal osteoma with a surrounding subretinal hemorrhage in the left eye (Fig 5). PATHOLOGIC FINDINGS
Histologic examination of the multiple skin excisions was characteristic for the linear sebaceous nevus of Jadassohn: papillomatous hyperplasia and hyperkeratosis of the epidermis with small, poorly formed pilosebaceous units and many mature closely packed sebaceous glands (Fig 6). Histologic findings of the conjunctival lesions showed focal cartilagenous choristomatous masses ensheathed in a fibrous pseudoperichondrium. Interspersed through the cartilagenous tissue were small, apparently ectopic lacrimal glands (Fig 7). No superior rectus muscle tissue was found. All tissues were well differentiated and cytologically benign.
Fig 4. CT scan. Bilateral choroidal osteomas.
DISCUSSION On reviewing the literature, 20 well-documented cases with follow-up adequate to determine the seizure status
Table 1. Cases Reviewed Reference
Case/Sex
Race
Seizures (age at onset)
Mental Retardation
Mardin and Venters 4 Mardin and Venters4 Monahan et al 5 Moynahan and Wol1l 6 Solomon et al 7 Solomon et al 7 Sugarman and Reed 8 Bianchine9 Herbst and Cohen 1O Lansky et alII Lansky et alII Holden and Dekaban 12 Holden and Dekaban 12 Lovejoy and Boyle13 Lovejoy and Boyle13 Mooriani and Shaw14 Campbell et al 15 Wilkes et al 16 Shochot 17
l/M 2/M 3/F 4/M 5/F 6/M 7/F 8/M 9/F 10/F 11/M 12/M 13/M 14/M 15/M 16/F 17/F 18/M 19/F 20/M
White Black White Not given Black White Asian White White White Black White White White White Asian Asian Black White White
Yes (7 wk) Yes (4 mol Yes (3 mol Yes (5 mol Yes (1 yr) No Yes (5 mol Yes (7 mol Yes (5 mol Yes (5 mol Yes (8 mol Yes (2 days) Yes (3 mol Yes (4 mol No Yes (6 mol Yes (7 mol No Death (1 wk) No
Mild Mild Severe Yes* Unknown None Severe Moderate Mild Death Severe Severe Severe Severe None Yes* Yes* None Death None
*Degree not stated.
280
LAMBERT, et al • LINEAR NEVUS
Fig S. Top, fundus photo of subretinal neovascular membrane with subretinal hemorrhage overlying choroidal osteoma of left eye (8 years of age). Fig 6. Bottom left, characteristic nevus of Jadassohn: papillomatous hyperplasia and hyperkeratosis of the epidermis with small, poorly formed pilosebaceous units and mature closely packed sebaceous glands (skin lesion). Fig 7. Bottom right, focal cartilagenous chorismatous masses ensheathed in a fibrous pseudoperichondrium with interspersed ectopic lacrimal glands (conjunctival lesion).
of each were found.4-17 Several previously reported cases were not included because of lack of adequate followUp,18 lack of adequate data,19 and lesions that were not facial and midline. 2o,21 Table 1 lists the patients, their sex, their race, and the presence or absence of seizures and mental retardation. The following is a summary of this group of 20 patients. (l) All skin lesions were present at birth.
(2) Seizures and EEG changes were not an absolute finding and were present in 16 of the 20 patients. Onset in all 16 patients occurred before age 1. Types of seizures included apneic spells, myoclonic seizures, psychomotor seizures, and Jacksonian and Grand Mal seizures. (3) Mental retardation varied from none to severe. The four patients without seizures were the same four that had normal intelligence. (4) There was no racial predilection. (5) There was no familial incidence. (6) There appeared to be no sex predilection. (7) Eye lesions reported include conjunctival lipodermoids, lid involvement of the nevus sebaceum,
colobomata of the lid, iris, choroid, and disc, and angiomas of the orbit. (8) Other associated abnormalities include unilateral cortical atrophy, hydrocephalus, vitamin D-resistant rickets, and cardiac abnormalities.
SUMMARY The linear nevus sebaceous syndrome as originally described included the triad of the characteristic midline facial lesion, seizures, and mental retardation. Our case and our review of the literature show that seizures and mental retardation are not an absolute finding and should not be assumed until present. We suggest that the syndrome should now be considered to be an oculoneurocutaneous syndrome with the triad of: (l) the midline facial linear nevus sebaceous of Jadassohn; (2) neurologic abnormalities, which may include cerebral and cerebellar hypoplasia, arachnoid cysts, seizures, EEG abnormalities, and mental retardation; and (3) ocular abnormalities, which may include conjunctival lipodermoids, colobo281
OPHTHALMOLOGY
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MARCH 1987 •
mata of the lids, iris, choroid, and disc, angiomas of the orbit, osseous choristomas of the choroid, and subretinal neovascularization.
REFERENCES 1. Van der Hoeve J. The Doyne memorial lecture: eye symptoms in phakomatoses. Trans Ophthalmol Soc UK 1932; 52:380. 2. Feuerstein RC, Mims LC. Linear nevus sebaceus with convulsions and mental retardation. Am J Dis Child 1962; 104:675-9. 3. Marks JG, Tomasovic JJ. Linear nevus sebaceus syndrome: a case report. JAm Acad Derm 1980; 2:31-2. 4. Marden PM, Venters HD Jr. A new neurocutaneous syndrome. Am J Dis Child 1966; 112:79-81. 5. Monahan RH, Hill CW, Venters HD. Multiple choristomas, convulsions and mental retardation as a new neurocutaneous syndrome. Am J Ophthalmol1967; 64:529-32. 6. Moynahan EJ, Wolff OH. A new neuro-cutaneous syndrome (skin, eye, and brain) consisting of linear naevus, bilateral lipo-dermoid of the conjunctivae, cranial thickening, cerebral cortical atrophy and mental retardation. Br J Derm 1967; 79:651-2. 7. Soloman LM, Fretzin OF, Dewald RL. The epidermal nevus syndrome. Arch Derm 1968; 97:273-85. 8. Sugarman GI, Reed WB. Two unusual neurocutaneous disorders with facial cutaneous signs. Arch Neuro11969; 21 :242-7. 9. Bianchine JW. The nevus sebaceous of Jadassohn: a neurocutaneous syndrome and a potentially premalignant lesion. Am J Dis Child 1970; 120:223-8. 10. Herbst BA, Cohen ME. Linea nevus sebaceus: a neurocutaneous
282
11.
12. 13. 14. 15.
16.
17.
18.
19. 20.
21.
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syndrome associated with infantile spasms. Arch Neurol 1971; 24: 317-22. Lansky LL, Funderburk S, Cuppage FE, Schimke RN, Diehl. Linear sebaceous nevus syndrome a hamartoma variant. AM. Am J Dis Child 1972; 123:587-90. Holden KR, Dekaban AS. Neurological involvement in nevus unis lateris and nevus linearis sebaceus. Neurology 1972; 22:879-87. Lovejoy FH, Boyle WE. Linear nevus sebaceous syndrome: report of two cases and a review of the literature. Pediatrics 1973; 52:382-7. Moorjani R, Shaw DG. Feuerstein and Mims syndrome with resistant rickets. Pediatr Radiol 1976; 5: 120-2. Campbell WW, Buda FB, Sorensen G. Linear nevus sebaceous syndrome: neurological aspects documented by brain scans correlated with developmental history and radiographic studies. Mil Med 1978; March 175-8. Wilkes SR, Campbell RJ, Waller RR. Ocular malformation in association with ipsilateral facial nevus of Jadassohn. Am J Ophthalmol 1981; 92:344-52. Shochot Y, Romano A, Barishak YR, Feinstein A, Brish M, Bimbaum E, Blumenthal M, Goodman RM. Eye findings in the linear sebaceous nevus syndrome: a possible clue to the pathogenesis. J Craniofac Genet Dev Bioi 1982; 2:289-94. Lantis S, Leyden J, Thew M, Heaton C. Nevus sebaceus of Jadassohn: part of a new neurocutaneous syndrome. Arch Derm 1968; 98:11723. Gellis SS, Feingold M. Picture of the month. Am J Dis Child 1970; 120:139-40. Lentz CL, Altman J, Mopper C. Nevus sebaceus of Jadassohn: report of a case with multiple and extensive lesions and an unusual linear distribution. Arch Derm 1968; 97:294-6. Marlowe FI, Cron C. Linear nevus sebaceous of Jadassohn. Laryngoscope 1979; 89:666-70.
Syndrome
H. MICHAEL LAMBERT, MD, JACK O. SIPPERLEY, MD, JOHN W. SHORE, MD, J. PAUL DIECKERT, MD, RICHARD EVANS, MD, DONALD K. LOWD, OT
Abstract: The linear nevus sebaceous syndrome is a rare disorder first described in 1962 in two cases by Feuerstein and Mims. As originally described, it consisted of the triad of the characteristic midline facial linear nevus sebaceous of Jadassohn, seizures, and mental retardation. The authors have followed a patient with this syndrome from birth to age 8 and although he does demonstrate several neurologic and ocular abnormalities, he enjoys normal intelligence and has never suffered seizures. The features of this syndrome and several new ocular findings demonstrated in this case are described. Based on our longterm follow-up of this patient and our review of the reported cases to date, the authors suggest that the triad for this disorder should be changed to include the midline facial linear nevus sebaceous of Jadassohn, neurologic abnormalities which may, but not necessarily, include seizures and mental retardation, and ophthalmologic abnormalities. [Key words: Feuerstein-Mims syndrome, linear nevus of Jadassohn, linear nevus sebaceum, phakomatosis, oculoneurocutaneous, osseous choristoma, subretinal neovascularization.] Ophthalmology 94:
278-282, 1987
In 1932, Van der Hoeve 1 first used the term "phakomatosis," meaning mother spot or birthmark, to describe the interrelationship of several congenital neurocutaneous syndromes including encephalotrigeminal angiomatosis (Sturge-Weber syndrome), neurofibromatosis (Von Recklinghausen's disease), tuberous sclerosis (Bourneville's disease), and angiomatosis of the retina and cerebellum (Von Hippel-Lindau disease). Ataxia-telangiectasia (Louis-Bar syndrome) was later added to this group. In 1963, Feuerstein and Mims2 described two patients with a new neurocutaneous disorder consisting of the triad of a characteristic midline facial skin lesion (linear sebaceous nevus of Jadassohn), seizures, and mental retardation. Since their original description, other cases of this syndrome with a growing list of associated lesions have been reported. We describe a case of the linear nevus seFrom the Department of Ophthalmology, Wilford Hall USAF Medical Center, San Antonio. The views expressed herein are those of the authors and do not necessarily reflect those of the US Air Force or the Department of Defense. Reprint requests to H. Michael Lambert, LtCoI, USAF, MC, Department of Ophthalmology (SGHSE), Wilford Hall USAF Medical Center, San Antonio, TX 78236.
278
baceous syndrome with 8 years of follow-up (previously described at the time of presentation3). Based on this case and a review of the literature, we suggest a modification of Feuerstein and Mims' original triad.
CASE REPORT In 1977, a 5-month-old white boy was referred to Wilford Hall USAF Medical Center for evaluation of an unusual facial lesion. He was the product of an uncomplicated term pregnancy and normal vaginal delivery. Family history was negative for any significant illnesses, skin lesions, seizures, or mental retardation. On physical examination, the patient was found to have a pale yellow plaque on the left side of his face extending from the scalp to the jaw and anteriorly to the midline. The lesion was smooth in appearance in the scalp area, becoming more verrucous in the area of the jaw. Marked alopecia was apparent in the left fronto-parietal region (Fig I). No other skin lesions were present. On neurologic examination, the patient was found to have normal psychomotor development with a head circumference at the 50th percentile. He had no history of seizures and had a
LAMBERT, et al • LINEAR NEVUS
Fig 1. Top leji and right. front and side views. The facial lesion extended from the scalp to the jaw and to the midline (age 5 months). The lesion was smooth in parietal region, becoming more verrucous in the area of the jaw. The left lid was notched and held in a narrowed position. There was marked alopecia of the fronto-parietal region. Fig 2. Bottom leji, CT shows mild atrophy of the left cerebral and cerebellar hemispheres and a large arachnoid cyst. Fig 3. Bottom right, conjunctival choristomatous lesions ofleft eye extending into cornea.
normal electroencephalogram (EEG). A computed tomography
(Cf) scan demonstrated moderate atrophy of the left cerebral
and cerebellar hemispheres and a large arachnoid cyst (Fig 2). On ophthalmologic examination, he was able to fix and follow with the right eye, but had poor fixation in the left. The left upper eyelid was notched, and the fissure was narrowed by the nevus sebaceum. The coloboma involved the puncta and canaliculus. Apparent lipodermoids were found in the superior and inferior conjunctiva of the left eye and in the superior cul-desac of the right (Fig 3). Ductions in the left eye were markedly
reduced with a left exotropia and hypotropia with the right eye fixating. Results offundus examination showed a disc coloboma in the left eye and bilateral "pale areas" superior to the disc in the right eye and surrounding the disc and extending into the temporal periphery in the left. Amblyopia therapy was instituted immediately but vision failed to improve in the left eye. Over the past 8 years, the patient has been followed jointly by plastic surgery, neurology, and ophthalmology. He has undergone serial excisions of his facial lesions, and hair-bearing tissue has been moved to the area of alopecia. The majority of
279
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the facial nevus has been removed. The patient has normal intelligence and has had no seizures. He does have a perplexing mild atrophy of the left extremities. Results of repeat fundus examinations have shown the "pale areas" to be yellow-white choroidal masses. In the right eye, the lesion now extends from the disc superiorly, and in the left eye the lesion surrounds the disc and extends temporally. cr scans of the orbits have confirmed the diagnosis of bilateral choroidal osteomas (Fig 4). At age 7, cosmetic repair of his left hypotropia and exotropia was attempted, but at surgery the superior rectus muscle was found to be absent. The remaining extraocular muscles were surrounded and infiltrated by fibro-fatty tissue, which was partially excised. Partial correction of his ptosis was attained by a silicone frontalis sling. The eyelid coloboma was closed primarily. At age 8, amacular subretinal neovascular membrane developed over the choroidal osteoma with a surrounding subretinal hemorrhage in the left eye (Fig 5). PATHOLOGIC FINDINGS
Histologic examination of the multiple skin excisions was characteristic for the linear sebaceous nevus of Jadassohn: papillomatous hyperplasia and hyperkeratosis of the epidermis with small, poorly formed pilosebaceous units and many mature closely packed sebaceous glands (Fig 6). Histologic findings of the conjunctival lesions showed focal cartilagenous choristomatous masses ensheathed in a fibrous pseudoperichondrium. Interspersed through the cartilagenous tissue were small, apparently ectopic lacrimal glands (Fig 7). No superior rectus muscle tissue was found. All tissues were well differentiated and cytologically benign.
Fig 4. CT scan. Bilateral choroidal osteomas.
DISCUSSION On reviewing the literature, 20 well-documented cases with follow-up adequate to determine the seizure status
Table 1. Cases Reviewed Reference
Case/Sex
Race
Seizures (age at onset)
Mental Retardation
Mardin and Venters 4 Mardin and Venters4 Monahan et al 5 Moynahan and Wol1l 6 Solomon et al 7 Solomon et al 7 Sugarman and Reed 8 Bianchine9 Herbst and Cohen 1O Lansky et alII Lansky et alII Holden and Dekaban 12 Holden and Dekaban 12 Lovejoy and Boyle13 Lovejoy and Boyle13 Mooriani and Shaw14 Campbell et al 15 Wilkes et al 16 Shochot 17
l/M 2/M 3/F 4/M 5/F 6/M 7/F 8/M 9/F 10/F 11/M 12/M 13/M 14/M 15/M 16/F 17/F 18/M 19/F 20/M
White Black White Not given Black White Asian White White White Black White White White White Asian Asian Black White White
Yes (7 wk) Yes (4 mol Yes (3 mol Yes (5 mol Yes (1 yr) No Yes (5 mol Yes (7 mol Yes (5 mol Yes (5 mol Yes (8 mol Yes (2 days) Yes (3 mol Yes (4 mol No Yes (6 mol Yes (7 mol No Death (1 wk) No
Mild Mild Severe Yes* Unknown None Severe Moderate Mild Death Severe Severe Severe Severe None Yes* Yes* None Death None
*Degree not stated.
280
LAMBERT, et al • LINEAR NEVUS
Fig S. Top, fundus photo of subretinal neovascular membrane with subretinal hemorrhage overlying choroidal osteoma of left eye (8 years of age). Fig 6. Bottom left, characteristic nevus of Jadassohn: papillomatous hyperplasia and hyperkeratosis of the epidermis with small, poorly formed pilosebaceous units and mature closely packed sebaceous glands (skin lesion). Fig 7. Bottom right, focal cartilagenous chorismatous masses ensheathed in a fibrous pseudoperichondrium with interspersed ectopic lacrimal glands (conjunctival lesion).
of each were found.4-17 Several previously reported cases were not included because of lack of adequate followUp,18 lack of adequate data,19 and lesions that were not facial and midline. 2o,21 Table 1 lists the patients, their sex, their race, and the presence or absence of seizures and mental retardation. The following is a summary of this group of 20 patients. (l) All skin lesions were present at birth.
(2) Seizures and EEG changes were not an absolute finding and were present in 16 of the 20 patients. Onset in all 16 patients occurred before age 1. Types of seizures included apneic spells, myoclonic seizures, psychomotor seizures, and Jacksonian and Grand Mal seizures. (3) Mental retardation varied from none to severe. The four patients without seizures were the same four that had normal intelligence. (4) There was no racial predilection. (5) There was no familial incidence. (6) There appeared to be no sex predilection. (7) Eye lesions reported include conjunctival lipodermoids, lid involvement of the nevus sebaceum,
colobomata of the lid, iris, choroid, and disc, and angiomas of the orbit. (8) Other associated abnormalities include unilateral cortical atrophy, hydrocephalus, vitamin D-resistant rickets, and cardiac abnormalities.
SUMMARY The linear nevus sebaceous syndrome as originally described included the triad of the characteristic midline facial lesion, seizures, and mental retardation. Our case and our review of the literature show that seizures and mental retardation are not an absolute finding and should not be assumed until present. We suggest that the syndrome should now be considered to be an oculoneurocutaneous syndrome with the triad of: (l) the midline facial linear nevus sebaceous of Jadassohn; (2) neurologic abnormalities, which may include cerebral and cerebellar hypoplasia, arachnoid cysts, seizures, EEG abnormalities, and mental retardation; and (3) ocular abnormalities, which may include conjunctival lipodermoids, colobo281
OPHTHALMOLOGY
•
MARCH 1987 •
mata of the lids, iris, choroid, and disc, angiomas of the orbit, osseous choristomas of the choroid, and subretinal neovascularization.
REFERENCES 1. Van der Hoeve J. The Doyne memorial lecture: eye symptoms in phakomatoses. Trans Ophthalmol Soc UK 1932; 52:380. 2. Feuerstein RC, Mims LC. Linear nevus sebaceus with convulsions and mental retardation. Am J Dis Child 1962; 104:675-9. 3. Marks JG, Tomasovic JJ. Linear nevus sebaceus syndrome: a case report. JAm Acad Derm 1980; 2:31-2. 4. Marden PM, Venters HD Jr. A new neurocutaneous syndrome. Am J Dis Child 1966; 112:79-81. 5. Monahan RH, Hill CW, Venters HD. Multiple choristomas, convulsions and mental retardation as a new neurocutaneous syndrome. Am J Ophthalmol1967; 64:529-32. 6. Moynahan EJ, Wolff OH. A new neuro-cutaneous syndrome (skin, eye, and brain) consisting of linear naevus, bilateral lipo-dermoid of the conjunctivae, cranial thickening, cerebral cortical atrophy and mental retardation. Br J Derm 1967; 79:651-2. 7. Soloman LM, Fretzin OF, Dewald RL. The epidermal nevus syndrome. Arch Derm 1968; 97:273-85. 8. Sugarman GI, Reed WB. Two unusual neurocutaneous disorders with facial cutaneous signs. Arch Neuro11969; 21 :242-7. 9. Bianchine JW. The nevus sebaceous of Jadassohn: a neurocutaneous syndrome and a potentially premalignant lesion. Am J Dis Child 1970; 120:223-8. 10. Herbst BA, Cohen ME. Linea nevus sebaceus: a neurocutaneous
282
11.
12. 13. 14. 15.
16.
17.
18.
19. 20.
21.
VOLUME 94
•
NUMBER 3
syndrome associated with infantile spasms. Arch Neurol 1971; 24: 317-22. Lansky LL, Funderburk S, Cuppage FE, Schimke RN, Diehl. Linear sebaceous nevus syndrome a hamartoma variant. AM. Am J Dis Child 1972; 123:587-90. Holden KR, Dekaban AS. Neurological involvement in nevus unis lateris and nevus linearis sebaceus. Neurology 1972; 22:879-87. Lovejoy FH, Boyle WE. Linear nevus sebaceous syndrome: report of two cases and a review of the literature. Pediatrics 1973; 52:382-7. Moorjani R, Shaw DG. Feuerstein and Mims syndrome with resistant rickets. Pediatr Radiol 1976; 5: 120-2. Campbell WW, Buda FB, Sorensen G. Linear nevus sebaceous syndrome: neurological aspects documented by brain scans correlated with developmental history and radiographic studies. Mil Med 1978; March 175-8. Wilkes SR, Campbell RJ, Waller RR. Ocular malformation in association with ipsilateral facial nevus of Jadassohn. Am J Ophthalmol 1981; 92:344-52. Shochot Y, Romano A, Barishak YR, Feinstein A, Brish M, Bimbaum E, Blumenthal M, Goodman RM. Eye findings in the linear sebaceous nevus syndrome: a possible clue to the pathogenesis. J Craniofac Genet Dev Bioi 1982; 2:289-94. Lantis S, Leyden J, Thew M, Heaton C. Nevus sebaceus of Jadassohn: part of a new neurocutaneous syndrome. Arch Derm 1968; 98:11723. Gellis SS, Feingold M. Picture of the month. Am J Dis Child 1970; 120:139-40. Lentz CL, Altman J, Mopper C. Nevus sebaceus of Jadassohn: report of a case with multiple and extensive lesions and an unusual linear distribution. Arch Derm 1968; 97:294-6. Marlowe FI, Cron C. Linear nevus sebaceous of Jadassohn. Laryngoscope 1979; 89:666-70.