Original Paper Eur Neurol 1992;32:83-85
Masayuki I keela3 Hiroshi Tsukagoshib Department of Neurology, Asahi General Hospital. Chiba, Japan; Department of Neurology. Tokyo Medical and Dental University. Tokyo, Japan
Vogt-Koyanagi-Harada Disease Presenting Meningoencephalitis Report of a Case with Magnetic Resonance Imaging
KeyWords
Abstract
Uveitis Meningoencephalitis Vogt-Koyanagi-Harada disease Magnetic resonance imaging
A 40-year-old Japanese woman, formerly diagnosed as having Vogt-Koya nagi-Harada disease (VKH). developed a consciousness disturbance. There were nuchal rigidity and mild right facial weakness. Ophthalmological find ings were compatible with VKH. Lumbar puncture revealed moderate pleocy tosis. MRI showed multiple focal lesions. This case verifies parenchymatous involvement of CNS in VKH.
Vogt-Koyanagi-Harada disease (VKH) is a rare disor der of unknown origin [1. 2]. Its manifestations include uveitis and meningitis. Integumentary involvement, such as vitiligo, poliosis and alopecia, is also common [2, 3]. It is well documented mainly in the Japanese literature and to a lesser degree in other countries. Its neurological mani festations arc poorly understood, and its pathophysiologyleaves much to be elucidated. We present a case of VKH showing meningoencephalitis and discuss the clinicopathological correlation.
Case Report A 40-year-old Japanese woman was admitted because of con sciousness disturbance. She was healthy until 10 years before admis sion. when she developed bilateral impairment of visual acuity. A diagnosis of VKH was established. Cerebrospinal fluid was reported to have shown pleocytosis. She recovered spontaneously and subse quently remained well. Two weeks before admission, she noted gen
Received: January 29. 1991 Accepted: April 26.1991
eral fatigue and fever. One week later, she became disoriented and confused and was brought to our hospital. Her temperature was 39.2 "C, pulse 120 per minute and blood pressure 140/80 mm Hg. There was no lymphadenopathy, vitiligo, poliosis alopecia, erythema nodosum or thrombophlebitis. Genitalia were normal. On neurologic examination, she responded only to nox ious stimuli. The neck was stiff. The cranial nerves were normal except for mild right supranuclear facial weakness. There seemed to be no other overt motor dysfunction. Ophthalmologic examination revealed depigmentation of the chorioidea and a few Dalen-Fuchs’ spots, compatible with VKH. There was no physical finding suggest ing Behyet’s disease, tuberculosis, or toxoplasmosis. The white cell count was 22,800 (neutrophils 79%). A lumbar puncture, under an initial pressure of 200 mm H?0, revealed a pleo cytosis of 297/mm3 (28% monocytes and 72% polymorphonuclear cells), 65 mg/dl glucose and 61 mg/dl protein. CSF and blood cul tures were negative. The following laboratory tests were all normal: FTA-ABS, PPD skin test, antinuclear antibodies, latex agglutination test for toxoplasma and skin puncture test. An electroencephalogram showed prominent diffuse delta activity. Auditory brainstem re sponse and short latency somatosensory evoked potential were nor mal. Head CT scan revealed a low density lesion in the anterior limb of the left internal capsule with no contrast enhancement (fig. 1). Mag netic resonance was performed with a 0.5-tesla superconduction
Dr, Masayuki Ikcda Wellcome Surgical Institute University of Glasgow Garscube Estate Bearsden Road. Glasgow G6I IQH (UK)
€> 1992 S. Karger AG. Basel 0014-3022/92/0322-0083 $2.75/0
Downloaded by: Univ. of California Santa Cruz 128.114.34.22 - 10/7/2017 2:43:40 AM
Introduction
Fig. 2. Axial Ti-wcightcd (2.000/I00) MRI. Abnormal increased signal intensity in the region of the head of the left caudate nucleus, the anterior limb of the left internal capsule, the left putamen, the left temporoparieto-occipital region and the convex of the left cerebral hemisphere (a). High intensity is also noted in the tegmentum and the cerebellum (b).
magnet (Toshiba, Japan). Images were produced by a spin echo (SE) technique. A multislice multiecho technique was utilized to obtain axial images with an echo delay time (TE) of 30 and 100 ms and a repetition time (TR) of 2.000 ms. On the Ti-wcighted or proton den sity axial images, focal lesions of increased signal intensity were noted in the region of the anterior limb of the left internal capsule, the left temporoparieto-occipital region, the convex of the left hemi sphere. the tegmentum and the cerebellum (fig. 2). Bilateral carotid and vertebral angiography was negative. The patient had HL.A-tvpes A2, A24. Bw54, Bw55. Cwl and DR4. Only with general supportive care did her clinical condition steadily improve. We did not give her steroids. Follow-up lumbar puncture also revealed a decrease in CSF pleocytosis to 17/mm3 finally. During the course of recovery, it was difficult to evaluate the neuropsychological state because of associated consciousness distur bance. She became afebrile, alert and free of neurological deficit. She was discharged on the 30th hospital day. Convalescent serum showed no significant increase in the titers of viral antibodies, including herpes simplex, herpes zoster, mumps, measles, echo, coxsackie- and Japanese encephalitis virus. MRI, done 2 months after the acute episode, when she lived a healthy life, still revealed abnor mal intensity in the left caudate nucleus.
Discussion
This is a case of VKIT with meningoencephalitis. The uveomeningoencephalitic syndrome is caused by miscel laneous disorders [4], In this case, typical ophthalmo scopic findings, including Dalen-Fuchs’ spots and pleocy
84
Ikeda/Tsukagoshi
tosis in CSF, support the diagnosis of VKF1 [4], Labora tory data suggest no other disease such as Behcet's dis ease, toxoplasmosis or tuberculosis. Moreover, HLA typ ing is also compatible with VKH [5]. The patient presented with febrile illness and devel oped consciousness disturbance. Radiological examina tion verified the involvement of CNS compatible with meningoencephalitis. Many of the neurological features of VKH, such as hemiparesis. conjugate deviation of the eyes, ataxia and spinal cord signs, suggest not only menin geal but also parenchymatous involvement [6-11], How ever, there are only a few reports describing the radiologi cal findings of the brain in VKH. Lubin et al. [9] and Hiraki et al. [10] described cases with various neurologi cal signs, but CT were normal in all cases. To our knowl edge, MRI in this disorder have been evaluated in only 1 case. Nitta and Takamori [11] reported a case of VKH with Wallenberg's syndrome. MRI revealed high intensity area mainly in the cerebellum. Vertebral angiography dis closed occlusion of the right posterior inferior cerebellar artery1. They postulated vasculitis and/or demyelination in the central nervous system in VKH. In our case, CT revealed a low density lesion only in the anterior limb of the left internal capsule, but MRI disclosed more wide spread lesions extending from the cerebral cortex and white matter to the brainstem. In contrast with their case.
Vogt-Koyanagi-Harada Disease
Downloaded by: Univ. of California Santa Cruz 128.114.34.22 - 10/7/2017 2:43:40 AM
Fig. 1. CT scan obtained on admission without contrast enhancement, demonstrat ing decreased attenuation of the anterior limb of the left internal capsule.
the distribution of'the lesion is not compatible with vascu lar origin in our case. Moreover, angiography was nega tive. Therefore, infarction is not likely. The variety of the location of the lesions appears like that of such myelin disorders as multiple sclerosis. However, the abnormal signal intensities on MRI involve not only the white but also the gray matter. The pathological character of the lesions is difficult to specify, but several findings suggest an encephalitis-like process. The clinical picture including consciousness dis turbance, EEG slowing, pleocytosis in CSF and the self
limited course in this case is similar to that of benign viral encephalitis. Ocular pathology in VKH consists of an inflammatory process with or without granuloma [2, 12], Very few reports are available concerning the pathology of the central nervous system. Some equivocal cases involve an inflammatory process [1], We conclude that parenchymatous involvement of the CNS in VKH may not be rare, and careful neurological evaluation should be performed in all cases of VKH. MRI is the preferable imaging technique for the detection of the lesion.
References 4 Nozik RA. Schlaegel TF Jr: Diagnostic ap proach and miscellaneous analyses: in Tessler HH (ed): Diseases of Uvea. Clinical Ophthal mology. Philadelphia, Harper & Row, 1987. vol 4, chap 37. 5 Ohno S: Immunogenetic studies on various ocular diseases. Acta Soc Ophthalmol Jpn 1979:83:1875-1906. 6 Pattison EM: Uvcomeningoencephalitic syn drome (Vogt-Kayanagi-Harada). Arch Neurol 1965:12:197-205. 7 Richl JL, Andrews JM: The uveomeningoencephalitic syndrome. Neurology 1966:16:603— 609. 8 Uthoff D. Christiani K, Trettin H: Uveo-Meningo-Encephalitis (Vogt-Koyanagi-HaradaSyndrom). Nervenarzt 1979:50:464-466.
9 Lubin Jr, Loewenstein Jl, Frederick AR Jn Vogt-Koyanagi-Harada syndrome with focal neurologic signs. Am J Ophthalmol 1981:91: 332-341. 10 Hiraki Y. Kuwasaki N. Shoji H. Kaji M. Kubosiro T: Each one case of Vogt-Koyanagi-Ha rada disease with vestibular and cerebellar ataxia and multible cranial nerve palsies. Clin Neurol (Tokyo) 1989:29:54-58. 11 Nitta E. Takamori M: Wallenberg’s syndrome in a case of Vogt-Koyanagi-Harada disease. Clin Neurol (Tokyo) 1989;29:505-508. 12 Char CC, Palestine AG. Kuwabara T. Nussenblatt RB: Immunopathologic study of VogtKoyanagi-Harada syndrome. Am J Ophthal mol ! 988:105:607—6 11.
85
Downloaded by: Univ. of California Santa Cruz 128.114.34.22 - 10/7/2017 2:43:40 AM
1 Manor RS: Vogl-Koyanagi-Harada syndrome and related diseases; in Vinken PJ, Bruvn GW (cds); Infections of the Nervous System. Part 2. Handbook of Clinieal Neurology. Amsterdam. North-Holland. 1978, vol 34. pp 513-544. 2 Inomala H. Kato M: Vogl-Kayanagi-Harada disease: in McKcndall RR (ed): Viral Disease. Handbook of Clinical Neurology. Amsterdam. Elsevier. 1989. vol 56. pp 6 11-626. 3 Snyder DA. Tessier HH: Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol 1980:90:6975.
Masayuki I keela3 Hiroshi Tsukagoshib Department of Neurology, Asahi General Hospital. Chiba, Japan; Department of Neurology. Tokyo Medical and Dental University. Tokyo, Japan
Vogt-Koyanagi-Harada Disease Presenting Meningoencephalitis Report of a Case with Magnetic Resonance Imaging
KeyWords
Abstract
Uveitis Meningoencephalitis Vogt-Koyanagi-Harada disease Magnetic resonance imaging
A 40-year-old Japanese woman, formerly diagnosed as having Vogt-Koya nagi-Harada disease (VKH). developed a consciousness disturbance. There were nuchal rigidity and mild right facial weakness. Ophthalmological find ings were compatible with VKH. Lumbar puncture revealed moderate pleocy tosis. MRI showed multiple focal lesions. This case verifies parenchymatous involvement of CNS in VKH.
Vogt-Koyanagi-Harada disease (VKH) is a rare disor der of unknown origin [1. 2]. Its manifestations include uveitis and meningitis. Integumentary involvement, such as vitiligo, poliosis and alopecia, is also common [2, 3]. It is well documented mainly in the Japanese literature and to a lesser degree in other countries. Its neurological mani festations arc poorly understood, and its pathophysiologyleaves much to be elucidated. We present a case of VKH showing meningoencephalitis and discuss the clinicopathological correlation.
Case Report A 40-year-old Japanese woman was admitted because of con sciousness disturbance. She was healthy until 10 years before admis sion. when she developed bilateral impairment of visual acuity. A diagnosis of VKH was established. Cerebrospinal fluid was reported to have shown pleocytosis. She recovered spontaneously and subse quently remained well. Two weeks before admission, she noted gen
Received: January 29. 1991 Accepted: April 26.1991
eral fatigue and fever. One week later, she became disoriented and confused and was brought to our hospital. Her temperature was 39.2 "C, pulse 120 per minute and blood pressure 140/80 mm Hg. There was no lymphadenopathy, vitiligo, poliosis alopecia, erythema nodosum or thrombophlebitis. Genitalia were normal. On neurologic examination, she responded only to nox ious stimuli. The neck was stiff. The cranial nerves were normal except for mild right supranuclear facial weakness. There seemed to be no other overt motor dysfunction. Ophthalmologic examination revealed depigmentation of the chorioidea and a few Dalen-Fuchs’ spots, compatible with VKH. There was no physical finding suggest ing Behyet’s disease, tuberculosis, or toxoplasmosis. The white cell count was 22,800 (neutrophils 79%). A lumbar puncture, under an initial pressure of 200 mm H?0, revealed a pleo cytosis of 297/mm3 (28% monocytes and 72% polymorphonuclear cells), 65 mg/dl glucose and 61 mg/dl protein. CSF and blood cul tures were negative. The following laboratory tests were all normal: FTA-ABS, PPD skin test, antinuclear antibodies, latex agglutination test for toxoplasma and skin puncture test. An electroencephalogram showed prominent diffuse delta activity. Auditory brainstem re sponse and short latency somatosensory evoked potential were nor mal. Head CT scan revealed a low density lesion in the anterior limb of the left internal capsule with no contrast enhancement (fig. 1). Mag netic resonance was performed with a 0.5-tesla superconduction
Dr, Masayuki Ikcda Wellcome Surgical Institute University of Glasgow Garscube Estate Bearsden Road. Glasgow G6I IQH (UK)
€> 1992 S. Karger AG. Basel 0014-3022/92/0322-0083 $2.75/0
Downloaded by: Univ. of California Santa Cruz 128.114.34.22 - 10/7/2017 2:43:40 AM
Introduction
Fig. 2. Axial Ti-wcightcd (2.000/I00) MRI. Abnormal increased signal intensity in the region of the head of the left caudate nucleus, the anterior limb of the left internal capsule, the left putamen, the left temporoparieto-occipital region and the convex of the left cerebral hemisphere (a). High intensity is also noted in the tegmentum and the cerebellum (b).
magnet (Toshiba, Japan). Images were produced by a spin echo (SE) technique. A multislice multiecho technique was utilized to obtain axial images with an echo delay time (TE) of 30 and 100 ms and a repetition time (TR) of 2.000 ms. On the Ti-wcighted or proton den sity axial images, focal lesions of increased signal intensity were noted in the region of the anterior limb of the left internal capsule, the left temporoparieto-occipital region, the convex of the left hemi sphere. the tegmentum and the cerebellum (fig. 2). Bilateral carotid and vertebral angiography was negative. The patient had HL.A-tvpes A2, A24. Bw54, Bw55. Cwl and DR4. Only with general supportive care did her clinical condition steadily improve. We did not give her steroids. Follow-up lumbar puncture also revealed a decrease in CSF pleocytosis to 17/mm3 finally. During the course of recovery, it was difficult to evaluate the neuropsychological state because of associated consciousness distur bance. She became afebrile, alert and free of neurological deficit. She was discharged on the 30th hospital day. Convalescent serum showed no significant increase in the titers of viral antibodies, including herpes simplex, herpes zoster, mumps, measles, echo, coxsackie- and Japanese encephalitis virus. MRI, done 2 months after the acute episode, when she lived a healthy life, still revealed abnor mal intensity in the left caudate nucleus.
Discussion
This is a case of VKIT with meningoencephalitis. The uveomeningoencephalitic syndrome is caused by miscel laneous disorders [4], In this case, typical ophthalmo scopic findings, including Dalen-Fuchs’ spots and pleocy
84
Ikeda/Tsukagoshi
tosis in CSF, support the diagnosis of VKF1 [4], Labora tory data suggest no other disease such as Behcet's dis ease, toxoplasmosis or tuberculosis. Moreover, HLA typ ing is also compatible with VKH [5]. The patient presented with febrile illness and devel oped consciousness disturbance. Radiological examina tion verified the involvement of CNS compatible with meningoencephalitis. Many of the neurological features of VKH, such as hemiparesis. conjugate deviation of the eyes, ataxia and spinal cord signs, suggest not only menin geal but also parenchymatous involvement [6-11], How ever, there are only a few reports describing the radiologi cal findings of the brain in VKH. Lubin et al. [9] and Hiraki et al. [10] described cases with various neurologi cal signs, but CT were normal in all cases. To our knowl edge, MRI in this disorder have been evaluated in only 1 case. Nitta and Takamori [11] reported a case of VKH with Wallenberg's syndrome. MRI revealed high intensity area mainly in the cerebellum. Vertebral angiography dis closed occlusion of the right posterior inferior cerebellar artery1. They postulated vasculitis and/or demyelination in the central nervous system in VKH. In our case, CT revealed a low density lesion only in the anterior limb of the left internal capsule, but MRI disclosed more wide spread lesions extending from the cerebral cortex and white matter to the brainstem. In contrast with their case.
Vogt-Koyanagi-Harada Disease
Downloaded by: Univ. of California Santa Cruz 128.114.34.22 - 10/7/2017 2:43:40 AM
Fig. 1. CT scan obtained on admission without contrast enhancement, demonstrat ing decreased attenuation of the anterior limb of the left internal capsule.
the distribution of'the lesion is not compatible with vascu lar origin in our case. Moreover, angiography was nega tive. Therefore, infarction is not likely. The variety of the location of the lesions appears like that of such myelin disorders as multiple sclerosis. However, the abnormal signal intensities on MRI involve not only the white but also the gray matter. The pathological character of the lesions is difficult to specify, but several findings suggest an encephalitis-like process. The clinical picture including consciousness dis turbance, EEG slowing, pleocytosis in CSF and the self
limited course in this case is similar to that of benign viral encephalitis. Ocular pathology in VKH consists of an inflammatory process with or without granuloma [2, 12], Very few reports are available concerning the pathology of the central nervous system. Some equivocal cases involve an inflammatory process [1], We conclude that parenchymatous involvement of the CNS in VKH may not be rare, and careful neurological evaluation should be performed in all cases of VKH. MRI is the preferable imaging technique for the detection of the lesion.
References 4 Nozik RA. Schlaegel TF Jr: Diagnostic ap proach and miscellaneous analyses: in Tessler HH (ed): Diseases of Uvea. Clinical Ophthal mology. Philadelphia, Harper & Row, 1987. vol 4, chap 37. 5 Ohno S: Immunogenetic studies on various ocular diseases. Acta Soc Ophthalmol Jpn 1979:83:1875-1906. 6 Pattison EM: Uvcomeningoencephalitic syn drome (Vogt-Kayanagi-Harada). Arch Neurol 1965:12:197-205. 7 Richl JL, Andrews JM: The uveomeningoencephalitic syndrome. Neurology 1966:16:603— 609. 8 Uthoff D. Christiani K, Trettin H: Uveo-Meningo-Encephalitis (Vogt-Koyanagi-HaradaSyndrom). Nervenarzt 1979:50:464-466.
9 Lubin Jr, Loewenstein Jl, Frederick AR Jn Vogt-Koyanagi-Harada syndrome with focal neurologic signs. Am J Ophthalmol 1981:91: 332-341. 10 Hiraki Y. Kuwasaki N. Shoji H. Kaji M. Kubosiro T: Each one case of Vogt-Koyanagi-Ha rada disease with vestibular and cerebellar ataxia and multible cranial nerve palsies. Clin Neurol (Tokyo) 1989:29:54-58. 11 Nitta E. Takamori M: Wallenberg’s syndrome in a case of Vogt-Koyanagi-Harada disease. Clin Neurol (Tokyo) 1989;29:505-508. 12 Char CC, Palestine AG. Kuwabara T. Nussenblatt RB: Immunopathologic study of VogtKoyanagi-Harada syndrome. Am J Ophthal mol ! 988:105:607—6 11.
85
Downloaded by: Univ. of California Santa Cruz 128.114.34.22 - 10/7/2017 2:43:40 AM
1 Manor RS: Vogl-Koyanagi-Harada syndrome and related diseases; in Vinken PJ, Bruvn GW (cds); Infections of the Nervous System. Part 2. Handbook of Clinieal Neurology. Amsterdam. North-Holland. 1978, vol 34. pp 513-544. 2 Inomala H. Kato M: Vogl-Kayanagi-Harada disease: in McKcndall RR (ed): Viral Disease. Handbook of Clinical Neurology. Amsterdam. Elsevier. 1989. vol 56. pp 6 11-626. 3 Snyder DA. Tessier HH: Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol 1980:90:6975.
