21. Denny-Brown D, Meyer JS, Hornstein S. The significance of perceptual rivalry resulting from parietal lobe lesion. Brain 1952;75:434-471 22. Bender MD. Extinction and precipitation of cutaneous sensation. Arch Neurol Psychiatry 1945;54:1-9 23. Bisiach E, Luzzatti C, Perani D. Unilateral neglect, representational schema and consciousness. Brain 1979;102:609-618 24. Bisiach E, Luzzatti C. TJnilateral neglect of representational space. Cortex 1978;14:129-133 25. Heilman KM, Bowers D, Coslett HB, et al. Directional hypokinesia: prolonged reaction times for leftward movements in patients with right hemisphere lesions and neglect. Neurology 1985;35:855-859 26. Barbieri C, De Renzi E. Patterns of neglect dissociation. Behav Neurol 1989;2:13-24 27. Zarit SH, Kahn RL. Impairment and adaptation in chronic disabilities: spatial inattention. J Nerv Ment Dis 1974;159:63-72
Positron Emission Tomography in Shy-Drager Syndrome Mohit H. Bhatt, MD, DM, Barry J. Snow, MBChB, FRACP, W. R. Wayne Martin, MD, FRCPC, Sandra Cooper, RN, and Donald B. Calne, DM, FRCP, FKCPC
motor neuron deficits El]. Parlunsonism is an important manifestation of SDS [l-31. With the availability of {'8F)6-fluoro-1-dopa (6-FD) and positron emission tomography (PET), the functional integrity of the nigrostriatal pathway can be studied in vivo. Striatal 6F D uptake has been shown to be depressed in presynaptic nigrostriatal dopaminergic disorders such as idiopathic parkinsonism [4-71. T h e purpose of this study was to evaluate presynaptic nigrostriatal function in parkinsonism of SDS by using 6-FD PET.
Patients and Methods Patient I A 52-year-old man had a 4-year history of limb rigidity, impotence, incontinence of urine, chronic constipation, and multiple syncopal attacks. On examination he had postural hypotension (2201130 supine, 70150 standing). Kgidity, bradgkinesia, hypomimia, and hand tremor were present. His voice was monotonous and hypophonic. He had brisk tendon reflexes and bilateral extensor plantar responses. He scored 35 on the Columbia scale, which measures overall motor deficits in parkinsonian patients. Head computed tomographic (CT) scan was normal. Levodopdcarbidopa (2501 25 mg four times daily) and bromocriptine (20 mdday) did not improve his parkinsonism. Patient 2
We studied the nigrostriatal dopaminergic pathway in 3 patients with Shy-Drager syndrome, by using positron emission tomography and ['8F]6-fluoro-l-dopa to determine whether their parkinsonism correlated with impaired functional integrity of the presynaptic nigrostriatal pathway. One patient had short duration of disease, mild parkinsonism, and a normal positron emission tomographic scan, suggesting pathological changes functionally distal to the nigrostriatal pathway. T w o patients with longer duration of disease had more severe parkinsonism and reduced ['*F}Gfluoro-1-dopa uptake, suggesting impaired nigrostriatal dopaminergic function with progression of Shy-Drager syndrome. Bhatt MH, Snow BJ, Martin WRW, Cooper S, Calne DB. Positron emission tomography in Shy-Drager syndrome. Ann Neurol 1990;28:101-103 Shy-Drager Syndrome (SDS) is characterized by autonomic failure with any combination of parkinsonism, pyramidal dysfunction, cerebellar ataxia, and lower
From the Belzberg Laboratory of Clinical Neuroscience, Division of Neurology, University of British Columbia, and UBCiTRIUMF PET Program, Vancouver, Canada. Received Nov 26, 1989, and in revised form Jan 9, 1990. Accepted for publication Jan 17, 1990. Address correspondence to Dr Calne, Department of Medicine, Division of Neurology, 221 1, Wesbrook Mall, Vancouver, British Columbia, V6T 1W5, Canada.
A 52-year-old woman had a 4-year history of unclear speech and bradykinesia She suffered multiple syncopal attacks. Two years after the onset of her disease she developed incontinence of urine and feces. On examination there was a postural drop in blood pressure (130190 supine, 80/60 standing). She had rigidity, marked bradykinesia, hypomimia, and a fine postural hand tremor. Her Columbia score was 53. She had emotional lability, an active jaw jerk, brisk tendon reflexes, and bilateral extensor plantar responses. Head CT scan and magnetic resonance imaging were normal. Levodopdcarbidopa (250125 mg four times daily) and bromocriptine (15 mg1day) did not help her.
Patient 3 A 72-year-old woman had a 6-month history of unsteady gait, multiple syncopal attacks, and incontinence of urine and feces. On examination, she had a postural drop in blood pressure (1801110 supine, 55/30 standing) with hypomimia, cogwheel rigidity, bradykinesia, and bilateral spasticity. Her Columbia score was 28. She could not walk tandem. Rectal examination revealed a gaping, patulous anus. Head scan was normal. Levodopdcarbidopa (250125 mg three times daily) was not helpful. There were 14 normal control subjects (age range, 22-80 yr; mean, 45.5 yr). They were asymptomatic, neurological examination was normal, and none was taking medication. The data collection scheme for both groups (control subjects and patients) was identical, and a standard protocol for PET scanning approved by the University of British Columbia Ethics Committee was followed.
Methods PET studies were performed with the University of British ColumbiaiTRIUMF positron emission transaxial tomog-
Copyright 0 1990 by the American Neurological Association
101
API
Fig 1. Representative ~18F}6~uoro-I -dopa positmn emisrion tomographic images of the 3 patients and I normal control subject for comparison. Figures on the gray scale refer to the regional concentration of radioactivity in counts per second per milliliter. These images represent 30 minutes of emission data acquired during the second hour after administration of the tracer.
raphy VI system, which simultaneously generates 7 axial slices with center-to-center separation of 14.4 mm, in planeimage resolution averaging 9.2 mm (full width half maximum) and average axial resolution of approximately 11 mm. Each patient and control received 2.0 to 3.5 mCi 6-FD 1 hour after oral administration of 100 mg carbidopa. All antiparkinsonian drugs were withdrawn the night before the scan. A transmission scan was performed with Germanium 68 before administration of the tracer to correct emission data for attenuation occurring in the head. Twelve sequential emission scans were performed, each scan lasting 10 minutes, following the tracer injection. Sequential arterial blood samples were drawn from an indwelling radial artery catheter starting at the time of tracer administration and continuing to the end of the scanning procedure. Total plasma radioactivity was determined in each blood sample and plasma 6-FD metabolites were analyzed. Emission scans were analyzed by using a graphic model to calculate the steady-state uptake rate constant for "F from blood to striatum, an index of the functional integrity of the nigrostriatal nerve endings {8}. Regions of interest were placed visually on all slices in which radioactivity in excess of nonstriatal background activity was evident in the vicinity of the striatum. These regions of interest were large enough to ensure that all striatal activity was included. Multiple tomographic levels were averaged together, thus using the whole striatum for analysis. The images shown in Figure 1 are representative slices. Patients 1 and 3 each had CT scans of the head, and Patient 2 had both CT and magnetic resonance imaging prior to the PET scan.
Results Because of the strong influence of age on the striatal6FD uptake rate constant [S), the constants for patients and controls have been plotted against age (Fig 2). Patients 1 and 2 had 6-FD uptake rate constants that were below the 99% confidence limits for the control 102 Annals of Neurology
Vol 2 8 No 1 July 1990
"'"
iO
3;
40
M
m
70
&a
Age (years)
Fig 2. The striatal ~8F)6-fEuoro-l -dopa uptake rate constant plotted as a function ofage for 3 patients with Shy-Drager syndrome and 14 nomzal control subjects. The regression line is for the nomzal controlsubjects (r = 0.64, p < 0.05). The broken and unbroken curved lines mark the 90% and 99% limits forprediction for individual values, respectively. (PI, P2,and P3 correspond to Patients I , 2, and 3, respectively.)
group. Patient 3 had a 6-FD uptake rate constant within the normal range. Representative PET images of the patients are shown in Figure 1, together with a normal scan for comparison.
Discussion PET is a sensitive tool with which to study the function of the dopaminergic nigrostriatal pathway in vivo. Asymptomatic presynaptic lesions in humans and monkeys have been detected 19, lo], and an age-related reduction in dopamine uptake has been found in normal human subjects [Sf. Parkinsonism in SDS could be due, principally, to either involvement of the nigrostriatal dopaminergic pathway or degeneration distal to this pathway. All of our patients had parkinsonism, but only Patients 1 and 2 had PET evidence of a lesion of the nigrostriatal pathway. Clinically, these patients were distinguishable from Patient 3 by their more severe parkinsonism and their longer duration of symptoms (4 pears for Patients 1 and 2 vs. 6 months for Patient 3). We infer that the combination of parkinsonism and normal 6-FD PET in Patient 3 suggests dysfunction distal to the nigrostriatal system. The lack of a therapeutic response to levodopa in all patients also suggests an early lesion distal to the nigrostriatal system. Other workers have proposed a receptor disorder in SDS after demonstrating decreased {3H]spiperone binding in the caudate nucleus at postmortem examination { 111. This finding is in accord with OUT general conclusion. Reduced dopamine uptake was found in all patients with multiple system atrophy studied by Brooks and colleagues {12], and
several postmortem studies have reported nigral degeneration f13, 141. This is in agreement with the severe parkinsonism and marked dopaminergic deficit in Patients 1 and 2. Taking all these observations together, the evidence suggests the following sequence of events in SDS. Pathways postsynaptic to the nigrostriatal projection are involved early, resulting in clinical parkinsonism, which responds poorly to levodopa. Nigrostriatal neurons become involved as SDS progresses, and parkinsonism becomes more severe. Further studies with PET, of presynaptic nigrostriatal function and postsynaptic dopamine receptor function in conjunction with careful clinical evaluations, should help resolve the issue of the relative importance of pathological findings presynaptic and postsynaptic to the striatal dopaminergic nerve endings.
M. Bhatt was partly supported by The Birla Smarak Kosh, India. This work was supported by the Dystonia Medical Foundation. We thank Poppy Schofield, Tom Ruth, Michael Adams, Kellie Hewitt, and SalmaJivan of the TRIUMWPET program for their help in this project.
References 1. Shy MG, Drager GA. A neurological syndrome associated w-ith orthostatic hypotension. Arch Neurol 1960;2:511-527 2. Thomas JE, Schirger A. Neurologic manifestations in idiopathic orthostatic hypotension. Arch Neurol 1963;8:204-208 3. Schatz IJ, Podolsky S, Frame G. Idiopathic orthostatic hypotension. JAMA 1963;186:537-540 4. Garnett ES, Firnau G, Nahmias C. Dopamine visualized in the basal ganglia of living man. Nature 1983;305:137-138 5. Leenders KL, Palmer AJ, Quinn N, et al. Brain dopamine metabolism in patients with Parkinson’s disease measured with positron emission tomography. J Neurol Neurosurg Psychiatry 1986;49:853-860 6. Martin WRW, Stoessl AJ, Adam MJ, et al. Positron emission tomography in Parkinson’s disease: glucose and dopa metabolism. In: Yahr MD, Bergmann KJ, eds. Parkinson’s disease. Advances in neurology, vol 45. New York: Raven Press, 1986195-98 7. Nahmias C, Garnett ES, Firnau G, Iang A. Striatal dopamine distribution in parkinsonian patients during life. J Neurol Sci 1985;69:223-230 8. Martin WRW, Palmer MR, Patlak CS, Calne DB. Nigrostriatal function in humans studied with positron emission tomography. Ann Neurol 1989;26:535-542 9. Calne DB, LangstonJW, iMarrin WRW, et al. Positron emission tomography after MPTP: observations relating to the cause of Parkinson’s disease. Nature 1985;3 17:246-248 10. Guttman M, Young VW, Kim S, et al. Asymptomatic striatal dopamine depletion: PET scans in unilateral MPTP monkeys. Synapse 1988;2:469-473 11. Quik M, Spokes EG, Mackay AVP, Bannister R. Alterations in [3H’Jspiperone binding in human caudate nucleus, substantia nigra and frontal cortex in the Shy-Drager syndrome and Parkinson’s disease. J N e w 1 Sci 1979;43:429-437 12. Brooks DJ, Salmon EP, Mathias CJ, et al. Correlations between clinical disability and the integrity of the dopaminergic system in
patients with multiple system atrophy and pure autonomic fadure. Neurology 1989;39(suppl 1):274 13. Graham JG, Oppenheimer D R Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry 1969;32:28-34 14. Thapedi IM, Ashenhurst EM, Rozdilsky B. Shy-Drager syndrome Report of an autopsied case. Neurology 197 1;21:26-32
Rhinocerebral Mucormycosis: Management and Survival After Carotid Occlusion Steven L. Galetta, MD,*J- Allan E. Wulc, MD,? Herbert I. Goldberg, MDJ Charles W. Nichols, MD,? and Joel S. Glaser, MDP
Rhinocerebral mucormycosis is a rare but often fatal fungal infection. We present 2 patients with mucormycosis complicated by internal carotid artery thrombosis. Magnetic resonance imaging was superior to computed tomography in localizing the pathological process. Subtotal resection of devitalized tissue and intravenous a m photericin B therapy resulted in a successful outcome for both patients. Prompt recognition of this disorder by using modern diagnostic and therapeutic modalities promises to improve survival rates. Galetta SL, Wulc AE, Goldberg HI, Nichols CW, Glaser JS. Rhinocerebral mucormycosis: management and survival after carotid occlusion. Ann Neurol 1770;28:103-103 ~
~
~~~
Mucormycosis is a fungal infection by the organisms in the genera Mucor, Absidia, and Rhizopus [I, 2). The fungus has a widespread distribution and may be found in air, soil, vegetable matter, and bread mold [3]. The five major clinical presentations of mucormycosis are rhinocerebral, disseminated, pulmonary, gastrointestinal, and cutaneous f41. The rhinocerebral form is the most common and most likely to prove fatal. Infection typically originates in the paranasal sinuses and then rapidly spreads into the orbit, cavernous sinus, and brain parenchyma. Once the carotid artery has been
From the Departments of *Neurology, ?Ophthalmology-The Scheie Eye Institute, and $Radiology, University of Pennsylvania School of Medicine, Philadelphia, PA; and the §Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, FL. Received Dec 15. 1989, and in revised form Jan 22, 1990. Accepted for publication Jan 25, 1990. Address correspondence to Dr Galetta, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104.
Copyright 0 1990 by the American Neurological Association 103
Positron Emission Tomography in Shy-Drager Syndrome Mohit H. Bhatt, MD, DM, Barry J. Snow, MBChB, FRACP, W. R. Wayne Martin, MD, FRCPC, Sandra Cooper, RN, and Donald B. Calne, DM, FRCP, FKCPC
motor neuron deficits El]. Parlunsonism is an important manifestation of SDS [l-31. With the availability of {'8F)6-fluoro-1-dopa (6-FD) and positron emission tomography (PET), the functional integrity of the nigrostriatal pathway can be studied in vivo. Striatal 6F D uptake has been shown to be depressed in presynaptic nigrostriatal dopaminergic disorders such as idiopathic parkinsonism [4-71. T h e purpose of this study was to evaluate presynaptic nigrostriatal function in parkinsonism of SDS by using 6-FD PET.
Patients and Methods Patient I A 52-year-old man had a 4-year history of limb rigidity, impotence, incontinence of urine, chronic constipation, and multiple syncopal attacks. On examination he had postural hypotension (2201130 supine, 70150 standing). Kgidity, bradgkinesia, hypomimia, and hand tremor were present. His voice was monotonous and hypophonic. He had brisk tendon reflexes and bilateral extensor plantar responses. He scored 35 on the Columbia scale, which measures overall motor deficits in parkinsonian patients. Head computed tomographic (CT) scan was normal. Levodopdcarbidopa (2501 25 mg four times daily) and bromocriptine (20 mdday) did not improve his parkinsonism. Patient 2
We studied the nigrostriatal dopaminergic pathway in 3 patients with Shy-Drager syndrome, by using positron emission tomography and ['8F]6-fluoro-l-dopa to determine whether their parkinsonism correlated with impaired functional integrity of the presynaptic nigrostriatal pathway. One patient had short duration of disease, mild parkinsonism, and a normal positron emission tomographic scan, suggesting pathological changes functionally distal to the nigrostriatal pathway. T w o patients with longer duration of disease had more severe parkinsonism and reduced ['*F}Gfluoro-1-dopa uptake, suggesting impaired nigrostriatal dopaminergic function with progression of Shy-Drager syndrome. Bhatt MH, Snow BJ, Martin WRW, Cooper S, Calne DB. Positron emission tomography in Shy-Drager syndrome. Ann Neurol 1990;28:101-103 Shy-Drager Syndrome (SDS) is characterized by autonomic failure with any combination of parkinsonism, pyramidal dysfunction, cerebellar ataxia, and lower
From the Belzberg Laboratory of Clinical Neuroscience, Division of Neurology, University of British Columbia, and UBCiTRIUMF PET Program, Vancouver, Canada. Received Nov 26, 1989, and in revised form Jan 9, 1990. Accepted for publication Jan 17, 1990. Address correspondence to Dr Calne, Department of Medicine, Division of Neurology, 221 1, Wesbrook Mall, Vancouver, British Columbia, V6T 1W5, Canada.
A 52-year-old woman had a 4-year history of unclear speech and bradykinesia She suffered multiple syncopal attacks. Two years after the onset of her disease she developed incontinence of urine and feces. On examination there was a postural drop in blood pressure (130190 supine, 80/60 standing). She had rigidity, marked bradykinesia, hypomimia, and a fine postural hand tremor. Her Columbia score was 53. She had emotional lability, an active jaw jerk, brisk tendon reflexes, and bilateral extensor plantar responses. Head CT scan and magnetic resonance imaging were normal. Levodopdcarbidopa (250125 mg four times daily) and bromocriptine (15 mg1day) did not help her.
Patient 3 A 72-year-old woman had a 6-month history of unsteady gait, multiple syncopal attacks, and incontinence of urine and feces. On examination, she had a postural drop in blood pressure (1801110 supine, 55/30 standing) with hypomimia, cogwheel rigidity, bradykinesia, and bilateral spasticity. Her Columbia score was 28. She could not walk tandem. Rectal examination revealed a gaping, patulous anus. Head scan was normal. Levodopdcarbidopa (250125 mg three times daily) was not helpful. There were 14 normal control subjects (age range, 22-80 yr; mean, 45.5 yr). They were asymptomatic, neurological examination was normal, and none was taking medication. The data collection scheme for both groups (control subjects and patients) was identical, and a standard protocol for PET scanning approved by the University of British Columbia Ethics Committee was followed.
Methods PET studies were performed with the University of British ColumbiaiTRIUMF positron emission transaxial tomog-
Copyright 0 1990 by the American Neurological Association
101
API
Fig 1. Representative ~18F}6~uoro-I -dopa positmn emisrion tomographic images of the 3 patients and I normal control subject for comparison. Figures on the gray scale refer to the regional concentration of radioactivity in counts per second per milliliter. These images represent 30 minutes of emission data acquired during the second hour after administration of the tracer.
raphy VI system, which simultaneously generates 7 axial slices with center-to-center separation of 14.4 mm, in planeimage resolution averaging 9.2 mm (full width half maximum) and average axial resolution of approximately 11 mm. Each patient and control received 2.0 to 3.5 mCi 6-FD 1 hour after oral administration of 100 mg carbidopa. All antiparkinsonian drugs were withdrawn the night before the scan. A transmission scan was performed with Germanium 68 before administration of the tracer to correct emission data for attenuation occurring in the head. Twelve sequential emission scans were performed, each scan lasting 10 minutes, following the tracer injection. Sequential arterial blood samples were drawn from an indwelling radial artery catheter starting at the time of tracer administration and continuing to the end of the scanning procedure. Total plasma radioactivity was determined in each blood sample and plasma 6-FD metabolites were analyzed. Emission scans were analyzed by using a graphic model to calculate the steady-state uptake rate constant for "F from blood to striatum, an index of the functional integrity of the nigrostriatal nerve endings {8}. Regions of interest were placed visually on all slices in which radioactivity in excess of nonstriatal background activity was evident in the vicinity of the striatum. These regions of interest were large enough to ensure that all striatal activity was included. Multiple tomographic levels were averaged together, thus using the whole striatum for analysis. The images shown in Figure 1 are representative slices. Patients 1 and 3 each had CT scans of the head, and Patient 2 had both CT and magnetic resonance imaging prior to the PET scan.
Results Because of the strong influence of age on the striatal6FD uptake rate constant [S), the constants for patients and controls have been plotted against age (Fig 2). Patients 1 and 2 had 6-FD uptake rate constants that were below the 99% confidence limits for the control 102 Annals of Neurology
Vol 2 8 No 1 July 1990
"'"
iO
3;
40
M
m
70
&a
Age (years)
Fig 2. The striatal ~8F)6-fEuoro-l -dopa uptake rate constant plotted as a function ofage for 3 patients with Shy-Drager syndrome and 14 nomzal control subjects. The regression line is for the nomzal controlsubjects (r = 0.64, p < 0.05). The broken and unbroken curved lines mark the 90% and 99% limits forprediction for individual values, respectively. (PI, P2,and P3 correspond to Patients I , 2, and 3, respectively.)
group. Patient 3 had a 6-FD uptake rate constant within the normal range. Representative PET images of the patients are shown in Figure 1, together with a normal scan for comparison.
Discussion PET is a sensitive tool with which to study the function of the dopaminergic nigrostriatal pathway in vivo. Asymptomatic presynaptic lesions in humans and monkeys have been detected 19, lo], and an age-related reduction in dopamine uptake has been found in normal human subjects [Sf. Parkinsonism in SDS could be due, principally, to either involvement of the nigrostriatal dopaminergic pathway or degeneration distal to this pathway. All of our patients had parkinsonism, but only Patients 1 and 2 had PET evidence of a lesion of the nigrostriatal pathway. Clinically, these patients were distinguishable from Patient 3 by their more severe parkinsonism and their longer duration of symptoms (4 pears for Patients 1 and 2 vs. 6 months for Patient 3). We infer that the combination of parkinsonism and normal 6-FD PET in Patient 3 suggests dysfunction distal to the nigrostriatal system. The lack of a therapeutic response to levodopa in all patients also suggests an early lesion distal to the nigrostriatal system. Other workers have proposed a receptor disorder in SDS after demonstrating decreased {3H]spiperone binding in the caudate nucleus at postmortem examination { 111. This finding is in accord with OUT general conclusion. Reduced dopamine uptake was found in all patients with multiple system atrophy studied by Brooks and colleagues {12], and
several postmortem studies have reported nigral degeneration f13, 141. This is in agreement with the severe parkinsonism and marked dopaminergic deficit in Patients 1 and 2. Taking all these observations together, the evidence suggests the following sequence of events in SDS. Pathways postsynaptic to the nigrostriatal projection are involved early, resulting in clinical parkinsonism, which responds poorly to levodopa. Nigrostriatal neurons become involved as SDS progresses, and parkinsonism becomes more severe. Further studies with PET, of presynaptic nigrostriatal function and postsynaptic dopamine receptor function in conjunction with careful clinical evaluations, should help resolve the issue of the relative importance of pathological findings presynaptic and postsynaptic to the striatal dopaminergic nerve endings.
M. Bhatt was partly supported by The Birla Smarak Kosh, India. This work was supported by the Dystonia Medical Foundation. We thank Poppy Schofield, Tom Ruth, Michael Adams, Kellie Hewitt, and SalmaJivan of the TRIUMWPET program for their help in this project.
References 1. Shy MG, Drager GA. A neurological syndrome associated w-ith orthostatic hypotension. Arch Neurol 1960;2:511-527 2. Thomas JE, Schirger A. Neurologic manifestations in idiopathic orthostatic hypotension. Arch Neurol 1963;8:204-208 3. Schatz IJ, Podolsky S, Frame G. Idiopathic orthostatic hypotension. JAMA 1963;186:537-540 4. Garnett ES, Firnau G, Nahmias C. Dopamine visualized in the basal ganglia of living man. Nature 1983;305:137-138 5. Leenders KL, Palmer AJ, Quinn N, et al. Brain dopamine metabolism in patients with Parkinson’s disease measured with positron emission tomography. J Neurol Neurosurg Psychiatry 1986;49:853-860 6. Martin WRW, Stoessl AJ, Adam MJ, et al. Positron emission tomography in Parkinson’s disease: glucose and dopa metabolism. In: Yahr MD, Bergmann KJ, eds. Parkinson’s disease. Advances in neurology, vol 45. New York: Raven Press, 1986195-98 7. Nahmias C, Garnett ES, Firnau G, Iang A. Striatal dopamine distribution in parkinsonian patients during life. J Neurol Sci 1985;69:223-230 8. Martin WRW, Palmer MR, Patlak CS, Calne DB. Nigrostriatal function in humans studied with positron emission tomography. Ann Neurol 1989;26:535-542 9. Calne DB, LangstonJW, iMarrin WRW, et al. Positron emission tomography after MPTP: observations relating to the cause of Parkinson’s disease. Nature 1985;3 17:246-248 10. Guttman M, Young VW, Kim S, et al. Asymptomatic striatal dopamine depletion: PET scans in unilateral MPTP monkeys. Synapse 1988;2:469-473 11. Quik M, Spokes EG, Mackay AVP, Bannister R. Alterations in [3H’Jspiperone binding in human caudate nucleus, substantia nigra and frontal cortex in the Shy-Drager syndrome and Parkinson’s disease. J N e w 1 Sci 1979;43:429-437 12. Brooks DJ, Salmon EP, Mathias CJ, et al. Correlations between clinical disability and the integrity of the dopaminergic system in
patients with multiple system atrophy and pure autonomic fadure. Neurology 1989;39(suppl 1):274 13. Graham JG, Oppenheimer D R Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry 1969;32:28-34 14. Thapedi IM, Ashenhurst EM, Rozdilsky B. Shy-Drager syndrome Report of an autopsied case. Neurology 197 1;21:26-32
Rhinocerebral Mucormycosis: Management and Survival After Carotid Occlusion Steven L. Galetta, MD,*J- Allan E. Wulc, MD,? Herbert I. Goldberg, MDJ Charles W. Nichols, MD,? and Joel S. Glaser, MDP
Rhinocerebral mucormycosis is a rare but often fatal fungal infection. We present 2 patients with mucormycosis complicated by internal carotid artery thrombosis. Magnetic resonance imaging was superior to computed tomography in localizing the pathological process. Subtotal resection of devitalized tissue and intravenous a m photericin B therapy resulted in a successful outcome for both patients. Prompt recognition of this disorder by using modern diagnostic and therapeutic modalities promises to improve survival rates. Galetta SL, Wulc AE, Goldberg HI, Nichols CW, Glaser JS. Rhinocerebral mucormycosis: management and survival after carotid occlusion. Ann Neurol 1770;28:103-103 ~
~
~~~
Mucormycosis is a fungal infection by the organisms in the genera Mucor, Absidia, and Rhizopus [I, 2). The fungus has a widespread distribution and may be found in air, soil, vegetable matter, and bread mold [3]. The five major clinical presentations of mucormycosis are rhinocerebral, disseminated, pulmonary, gastrointestinal, and cutaneous f41. The rhinocerebral form is the most common and most likely to prove fatal. Infection typically originates in the paranasal sinuses and then rapidly spreads into the orbit, cavernous sinus, and brain parenchyma. Once the carotid artery has been
From the Departments of *Neurology, ?Ophthalmology-The Scheie Eye Institute, and $Radiology, University of Pennsylvania School of Medicine, Philadelphia, PA; and the §Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, FL. Received Dec 15. 1989, and in revised form Jan 22, 1990. Accepted for publication Jan 25, 1990. Address correspondence to Dr Galetta, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104.
Copyright 0 1990 by the American Neurological Association 103
