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Neurosarcoidosis causing ventricular loculation, hydrocephalus, and death
Surg Neurol 1986;26:67-71
67
Neurosarcoidosis Causing Ventricular Loculation, Hydrocephalus, and Death Michael Schlitt, M.D., Ernest R. Duvall, M.D., Jose Bonnin, M.D., and Richard B. Morawetz, M.D. Division of Neurosurgery and Departments of Radiology and Pathology, The University of Alabama School of Medicine, Birmingham, Alabama
Schlitt M, Duvall ER, BonninJ, Morawetz RB. Neurosarcoidosis causing ventricular loculation, hydrocephalus, and death. Surg Neurol 1986;26:67-71.
Ten years after a diagnosis of sarcoidosis, a 33-year-old woman presented with a severe headache of 5 days' duration. Neuroradiologic evaluation revealed a large cystic lesion of the left temporal lobe, causing a mass effect. An exploratory operation proved the lesion to be a loculated portion of the temporal horn of the lateral ventricle. Drainage of the loculated ventricle relieved the patient's cephalgia. Within 2 months, however, pain in the head recurred and an unsteady, broad-based gait appeared. Reevaluation disclosed hydrocephalus for which a ventriculoperitoneal shunt was inserted. After this procedure, the patient did well neurologically for 1 year, after which seizures, personality changes, incontinence, and disturbance of gait developed. Death occurred after revision of the shunt, and widespread granulomatous disease was found at autopsy. Neurosarcoidosis, with emphasis on intracranial mass lesions in sarcoidosis, is discussed; the role of surgical treatment in some of these lesions, and in hydrocephalus, is stressed. KEY WORDS: Hydrocephalus; Neurosarcoidosis; Ventricular loculation
Sarcoidosis is a common, chronic granulomatous disease, the etiology of which remains uncertain [8]. Neurologic manifestations occur in about 5% of patients with this disease [4], resulting in involvement of the central nervous system, peripheral nervous system, muscle, or a combination of these. Sarcoidosis of the central nervous system is associated with a poor prognosis; fewer than 25% of patients will go into remission [9]. Because of involvement of the cranial nerves, basal meninges,
Address reprint requests to: Michael Schlitt, M.D., Department of Neurosurgery, Mastin 714, 2451 FiUingimStreet, Mobile, Alabama 36617.
© 1986 by ElsevierSciencePublishingCo., Inc.
and hypothalamic-pituitary axis, disabling cranial nerve palsies, hydrocephalus, and endocrine abnormalities occur frequently. Treatment for intracranial neurosarcoidosis is controversial; steroids, surgical treatment, and cranial irradiation have been used [4-6,10]. A recent case at the University of Alabama at Birmingham illustrates involvement of the central nervous system. Entrapment of one portion of the ventricular system was followed closely by hydrocephalus. Surgical therapy resulted in good neurological recovery for 1 year, after which the patient gradually deteriorated and died as a result of her disease.
Case R e p o r t A 33-year-old dextral woman presented to the University of Alabama at Birmingham in May 1983 for evaluation of severe pain in the head. This pain had been intermittent for 2 months and then continuous for the 5 days prior to admission. The pain had originally been occipital but then had involved the whole head. At the time of her presentation it was sharp and unbearable. During the physical examination, the patient lost consciousness and was incontinent of urine. A computed tomography (CT) scan was performed, demonstrating a large low density area in the left temporal lobe (Figure 1), moderate left to right displacement of midline structures, and enlargement of the fourth ventricle. Sarcoidosis had been considered 10 years earlier when the patient presented with arthralgia, and the diagnosis had been confirmed by conjunctival biopsy. Prednisone had been administered for 9 months, and then gradually discontinued. System review revealed brief episodes of weakness of her right leg for 1 year. Neurologic examination disclosed a left nystagmus on horizontal gaze to that side, a moderate upper motor neuron right facial weakness, and a minimal pronator drift of the outstretched right arm. General physical examination revealed a papular pink rash over the lateral aspect of each nostril. A preoperative diagnosis of cystic neoplasm was 0090-3019/86/$3.50
68
Surg Neurol 1986;26:67-71
Figure 1. Admission computed tomography scan with contrast enhancement. (A) Enlarged fourth ventricle. Note irregular enhancement in perimesencephalic cisterns. (B) Cystic mass in left temporal lobe. Retrospectively.. configuration of the anterior margin of the lesion is typical for trapped temporal horn. (C) Edema in white matter of temporoparietal area. Enhancement superior to edema.
made, and an operation was planned. Dexamethasone therapy was begun. On May 27, 1983, exploration was undertaken through a left temporal craniotomy. Intracranial pressure was increased, and the middle temporal gyrus was enlarged. A cannula was passed into the lesion, and crystal-clear, colorless fluid was aspirated. The lesion was then entered via an incision placed anteriorly in the middle temporal gyrus. Smooth, glistening tissue lined the cavity; no tumor was visualized. A tuft of choroid plexus was attached to the medial wall of the cyst. To assist in the circulation of the cerebrospinal fluid (CSF), the tela choroidea suspending the choroid plexus was incised, and the wound closed. The postoperative diagnosis was loculated temporal horn of the left lateral ventricle due to sarcoid ependymitis. The postoperative course was uncomplicated. The pain in the head ceased, and the right-sided pronator drift resolved completely. The facial weakness improved but did not disappear, and the nystagmus persisted. Administration of dexamethasone was reduced rapidly, and the patient was discharged on the fifth postoperative day. After satisfactory convalescence for 1 month, the patient's headaches recurred and led to readmission. N o
Schlitt et al
new neurologic findings were present. A repeat CT scan demonstrated a decrease in the size of the cyst, and moderately enlarged third and fourth ventricles (Figure 2). The patient's headaches improved without intervention. Clinical observation 1 month later disclosed occasional headaches, as well as a broad-based and unsteady gait, tending toward the left. Early papilledema was present bilaterally. Incontinence and memory loss were denied. The nasal rash was absent. A repeat CT scan revealed a severe hydrocephalus. A right-sided ventriculoperitoneal shunt was inserted on August 2; intraoperative ventriculography and subsequent CT scanning confirmed communication of all chambers (Figure 3). Postoperative improvement was rapid, and the patient was discharged 6 days after her operation. Subsequent CT scans showed persisting enlargement of the fourth and left lateral ventricles. Neurologic examination 1 year after shunting revealed only the left nystagmus on left lateral gaze, mild asymmetry of the nasiolabial folds, and slightly diminished associated swing of the right arm during brisk walking. The nasal lesions had recurred, and millet-shaped granulomas surrounded the medial canthus of either eye. The patient was taking no medication and had returned to her former job. One month later she was brought to the emergency room after a tonic seizure. H e r family had noted listlessness, forgetfulness, and depression during the preceding month. N o new neurologic findings were noted; phenytoin and prednisone were prescribed. Two months later she returned to the emergency room. Family members related increasingly frequent seizures, urinary incontinence, and an inability to walk dur-
Neurosarcoidosis and Hydrocephalus
ing the preceding month. Compliance with prescribed medication had been poor. Neurologic examination disclosed gaze-evoked nystagmus to left and right and on upward gaze, and inability to stand or walk without support, as well as confusion and the preexisting facial asymmetry. A CT scan again revealed enlargement of the left lateral ventricle (Figure 4). On December 24, 1984, a ventricular catheter was placed in the left lateral ventricle and anastomosed to
Surg Neurol 1986;26:67-71
69
Figure 2. Computed tomography (CT) scan with contrast medium 36 days after initial admission. (A) Fourth ventricle is larger than on initial C T scan. (B) Left temporal horn is markedly smaller than on initial C T scan. The third ventricle has enlarged prominently. (C) Lateral ventricles are distinctly larger than on initial C T scan. Figure 3. Computed tomography scan after intraoperative injection of metrizamide into the fourth ventricle. (A) Metrizamide surrounds the cervicomedullary area. (B) Metrizamide in fourth ventricle (note layering). Slight contrast is visible in the pontocerebellar cisterns. Contrast not present in temporal horns. (C) Metrizamide in third and lateral ventricles.
70
Surg Neurol 1986;26:67-71
Figure 4. Computed tomography (CT~ scan with contrast l 7 months after initial C T scan, (A) Size of fourth ventricle is similar to that in prerious C T scan. Pathologicalenhancement is seen in hypothalamic area. (B) Third ventricle is enlarged but smaller than on 36th-day C T scan. Pathological enhancement adjacent to foramen on Monro. (C) Shunt in small right lateral ventricle. Enlargement of entire left latera/ ventricle.
the existing shunt proximal to the valve. After the procedure the patient's confusion partially resolved. On the third postoperative day, she complained suddenly of shortness of breath and progressed rapidly to cardiorespiratory arrest. Resuscitation attempts were unsuccessful. General autopsy demonstrated widespread granulomatous disease with severe involvement of the lungs, liver, spleen, and lymph nodes; the cardiac conduction system was uninvolved. There was no evidence of pulmonary thromboembolism. The ventriculoperitoneal shunt was patent. Examination of the brain and spinal cord disclosed severe involvement of the basal meninges, hypothalamus, pituitary stalk and gland, cranial and spinal nerves, choroid plexuses, and ventricular surfaces with noncaseating granulomas in various stages of evolution (Figure 5). At the level of the fourth ventricle, the ependymal involvement penetrated the medulla oblongata to a depth of 1 cm (Figure 6). The foramina of Luschka and Magendie were obliterated. In the spinal cord, the lumbosacral segments were more markedly involved.
Discussion Previously reported mass lesions in neurosarcoidosis have been parenchymal tumors, excrescences from basal
Schlitt et al
meninges and choroid plexuses, or meningocerebral infiltrates [3,4,6,7,10,11]. A loculated portion of the ventricular system presenting as a mass lesion has been reported twice [1,2]. Several reports [4,6,10] have suggested steroids as the mainstay of therapy in neurosarcoid mass lesions. Steroids were used only in an adjunctive manner in the treatment of the loculated ventricle in the present case. Because no egress from the trapped ventricle existed prior to operation, steroid medication would not have been expected to effect resolution. Computed tomography scans demonstrated adequate control of this aspect of the patient's disease, as manifested by lack of mass effect of the temporal horn of the lateral ventricle. Hydrocephalus complicating neurosarcoidosis is becoming more frequently recognized with the availability of CT scanning [1,2]. Prior to CT, hydrocephalus was not always recognized as the cause of intracranial hypertension, and patients often became blind or bedbound, or died after exploration of the posterior fossa [4]. More recently, several patients have been shunted; long-term outcome has varied from death to good neurological recoveries [4]. Hydrocephalus in neurosarcoidosis can be caused by granulomas in the third ventricle, aqueduct of Sylvius, or fourth ventricular outlets or by obstruction of the basal meninges [3,7,11]. Pathological evidence of defects in CSF reabsorption at the arachn0id villi has not been described. The cause of hydrocephalus in this case was obstruction of the outlets of the fourth ventricle and obliteration of the basal cisterns, as metrizamide injected into the fourth ventricle proved communication of all chambers at the time of the original placement of a shunt. Later, progressive
Neurosarcoidosis and Hydrocephalus
Surg Neurol 1986;26:67-71
71
Figure 6. Masses of granulomatous tissue filling the caudal angle of the fourth ventricle and infiltrating its floor.
Figure 5. Multiple confluent granulomas replacing most of the normal Hssue in the floor of the third ventricle. The asymmetry of the lateral ventricles is in part due to the adhesions of the ventricular walls produced by the granulomas.
granulomatous ependymitis isolated the right lateral ventricle from the remainder of the ventricular system, necessitating a left lateral ventricular catheter. The patient's compliance with the prescribed steroid therapy was uncertain and may have adversely influenced her outcome. Even so, histologic examination proved granulomas at various stages of evolution, suggesting that steroid administration could not have been expected to effect complete resolution of her neurosarcoidosis. The cause of death is uncertain but it is suspected that invasion of the medulla oblongata by the granu!omatous process may have produced some disturbances in the function of respiratory centers in the floor of the fourth ventricle, in contrast, despite virtually complete replacement of the hypothalamus and pituitary stalk and gland with sarcoid granulomas, the patient manifested no clinical signs of endocrine deficiency. Sarcoidosis is a chronic disease with protean systemic and neurologic manifestations. Lifelong observation with detailed neurologic examinations is required. Even so, data suggest neuromuscular involvement in more patients than is suspected clinically [4]. A high index of
suspicion of neurologic disease is therefore warranted regarding these patients, Serial CT scans should be performed, if intracranial pathology is suspected. Several different mass lesions and hydrocephalus can occur in neurosarc0idosis, necessitating surgical and medical management for optimal patient outcome. References 1. Bahr AL, Krumholz A, Kristt D, Hodges FJ III. Neuroradiological manifestations of intracranial sarcoidosis. Neuroradiology 1978;127:713-7. 2. Brooks BS, Gammal TE, Hungerford GD, Acker J, Trevor RP, Russell W. Radiologic evaluation of neurosarcoidosis: role of computed tomography. AJNR 1982;3:513-21. 3. Colover J. Sarcoidosis with involvement of the nervous system. Brain 1948;71:451-75. 4. Deianey P. Neurologic manifestations in sarcoidosis. Review of the literature, with a report of twenty-three cases. Ann Intern Med 1977;87:336-45. 5. Grizzanti JN, Knapp AB, Schecter AJ, Williams MH Jr. Treatment of sarcoid meningitis with radiotherapy. Am J Med 1982;73:605-8. 6. Healton EB, Zito G, Chauhan P, BrustJCM. Intracranial subdural sarcoid granuloma. Case report. J Neurosurg 1982;56:728-31. 7. Herring AB, Urich H. Sarcoidosis of the central nervous system. J Neurol Sci 1969;9:405-22. 8. James DG. Sarcoidosis. In: Hoeprich PD, ed. Infectious diseases. 2nd ed. Hagerstown, Maryland: Harper & Row, 1977:1203-9. 9. Neville E, Walker AN, James DG. Prognostic factors predicting outcome of sarcoidosis: an analysis of 818 cases. Q J Med 1983;52:525-33. 10. Powers WJ, Miller EM. Sarcoidosismimickingglioma: case report and review of intracranial sarcoid mass lesions. Neurology 1981;31:907-10. 11. Urich H. Neurological manifestations of sarcoidosis. Practitioner 1969;202:632-6.
67
Neurosarcoidosis Causing Ventricular Loculation, Hydrocephalus, and Death Michael Schlitt, M.D., Ernest R. Duvall, M.D., Jose Bonnin, M.D., and Richard B. Morawetz, M.D. Division of Neurosurgery and Departments of Radiology and Pathology, The University of Alabama School of Medicine, Birmingham, Alabama
Schlitt M, Duvall ER, BonninJ, Morawetz RB. Neurosarcoidosis causing ventricular loculation, hydrocephalus, and death. Surg Neurol 1986;26:67-71.
Ten years after a diagnosis of sarcoidosis, a 33-year-old woman presented with a severe headache of 5 days' duration. Neuroradiologic evaluation revealed a large cystic lesion of the left temporal lobe, causing a mass effect. An exploratory operation proved the lesion to be a loculated portion of the temporal horn of the lateral ventricle. Drainage of the loculated ventricle relieved the patient's cephalgia. Within 2 months, however, pain in the head recurred and an unsteady, broad-based gait appeared. Reevaluation disclosed hydrocephalus for which a ventriculoperitoneal shunt was inserted. After this procedure, the patient did well neurologically for 1 year, after which seizures, personality changes, incontinence, and disturbance of gait developed. Death occurred after revision of the shunt, and widespread granulomatous disease was found at autopsy. Neurosarcoidosis, with emphasis on intracranial mass lesions in sarcoidosis, is discussed; the role of surgical treatment in some of these lesions, and in hydrocephalus, is stressed. KEY WORDS: Hydrocephalus; Neurosarcoidosis; Ventricular loculation
Sarcoidosis is a common, chronic granulomatous disease, the etiology of which remains uncertain [8]. Neurologic manifestations occur in about 5% of patients with this disease [4], resulting in involvement of the central nervous system, peripheral nervous system, muscle, or a combination of these. Sarcoidosis of the central nervous system is associated with a poor prognosis; fewer than 25% of patients will go into remission [9]. Because of involvement of the cranial nerves, basal meninges,
Address reprint requests to: Michael Schlitt, M.D., Department of Neurosurgery, Mastin 714, 2451 FiUingimStreet, Mobile, Alabama 36617.
© 1986 by ElsevierSciencePublishingCo., Inc.
and hypothalamic-pituitary axis, disabling cranial nerve palsies, hydrocephalus, and endocrine abnormalities occur frequently. Treatment for intracranial neurosarcoidosis is controversial; steroids, surgical treatment, and cranial irradiation have been used [4-6,10]. A recent case at the University of Alabama at Birmingham illustrates involvement of the central nervous system. Entrapment of one portion of the ventricular system was followed closely by hydrocephalus. Surgical therapy resulted in good neurological recovery for 1 year, after which the patient gradually deteriorated and died as a result of her disease.
Case R e p o r t A 33-year-old dextral woman presented to the University of Alabama at Birmingham in May 1983 for evaluation of severe pain in the head. This pain had been intermittent for 2 months and then continuous for the 5 days prior to admission. The pain had originally been occipital but then had involved the whole head. At the time of her presentation it was sharp and unbearable. During the physical examination, the patient lost consciousness and was incontinent of urine. A computed tomography (CT) scan was performed, demonstrating a large low density area in the left temporal lobe (Figure 1), moderate left to right displacement of midline structures, and enlargement of the fourth ventricle. Sarcoidosis had been considered 10 years earlier when the patient presented with arthralgia, and the diagnosis had been confirmed by conjunctival biopsy. Prednisone had been administered for 9 months, and then gradually discontinued. System review revealed brief episodes of weakness of her right leg for 1 year. Neurologic examination disclosed a left nystagmus on horizontal gaze to that side, a moderate upper motor neuron right facial weakness, and a minimal pronator drift of the outstretched right arm. General physical examination revealed a papular pink rash over the lateral aspect of each nostril. A preoperative diagnosis of cystic neoplasm was 0090-3019/86/$3.50
68
Surg Neurol 1986;26:67-71
Figure 1. Admission computed tomography scan with contrast enhancement. (A) Enlarged fourth ventricle. Note irregular enhancement in perimesencephalic cisterns. (B) Cystic mass in left temporal lobe. Retrospectively.. configuration of the anterior margin of the lesion is typical for trapped temporal horn. (C) Edema in white matter of temporoparietal area. Enhancement superior to edema.
made, and an operation was planned. Dexamethasone therapy was begun. On May 27, 1983, exploration was undertaken through a left temporal craniotomy. Intracranial pressure was increased, and the middle temporal gyrus was enlarged. A cannula was passed into the lesion, and crystal-clear, colorless fluid was aspirated. The lesion was then entered via an incision placed anteriorly in the middle temporal gyrus. Smooth, glistening tissue lined the cavity; no tumor was visualized. A tuft of choroid plexus was attached to the medial wall of the cyst. To assist in the circulation of the cerebrospinal fluid (CSF), the tela choroidea suspending the choroid plexus was incised, and the wound closed. The postoperative diagnosis was loculated temporal horn of the left lateral ventricle due to sarcoid ependymitis. The postoperative course was uncomplicated. The pain in the head ceased, and the right-sided pronator drift resolved completely. The facial weakness improved but did not disappear, and the nystagmus persisted. Administration of dexamethasone was reduced rapidly, and the patient was discharged on the fifth postoperative day. After satisfactory convalescence for 1 month, the patient's headaches recurred and led to readmission. N o
Schlitt et al
new neurologic findings were present. A repeat CT scan demonstrated a decrease in the size of the cyst, and moderately enlarged third and fourth ventricles (Figure 2). The patient's headaches improved without intervention. Clinical observation 1 month later disclosed occasional headaches, as well as a broad-based and unsteady gait, tending toward the left. Early papilledema was present bilaterally. Incontinence and memory loss were denied. The nasal rash was absent. A repeat CT scan revealed a severe hydrocephalus. A right-sided ventriculoperitoneal shunt was inserted on August 2; intraoperative ventriculography and subsequent CT scanning confirmed communication of all chambers (Figure 3). Postoperative improvement was rapid, and the patient was discharged 6 days after her operation. Subsequent CT scans showed persisting enlargement of the fourth and left lateral ventricles. Neurologic examination 1 year after shunting revealed only the left nystagmus on left lateral gaze, mild asymmetry of the nasiolabial folds, and slightly diminished associated swing of the right arm during brisk walking. The nasal lesions had recurred, and millet-shaped granulomas surrounded the medial canthus of either eye. The patient was taking no medication and had returned to her former job. One month later she was brought to the emergency room after a tonic seizure. H e r family had noted listlessness, forgetfulness, and depression during the preceding month. N o new neurologic findings were noted; phenytoin and prednisone were prescribed. Two months later she returned to the emergency room. Family members related increasingly frequent seizures, urinary incontinence, and an inability to walk dur-
Neurosarcoidosis and Hydrocephalus
ing the preceding month. Compliance with prescribed medication had been poor. Neurologic examination disclosed gaze-evoked nystagmus to left and right and on upward gaze, and inability to stand or walk without support, as well as confusion and the preexisting facial asymmetry. A CT scan again revealed enlargement of the left lateral ventricle (Figure 4). On December 24, 1984, a ventricular catheter was placed in the left lateral ventricle and anastomosed to
Surg Neurol 1986;26:67-71
69
Figure 2. Computed tomography (CT) scan with contrast medium 36 days after initial admission. (A) Fourth ventricle is larger than on initial C T scan. (B) Left temporal horn is markedly smaller than on initial C T scan. The third ventricle has enlarged prominently. (C) Lateral ventricles are distinctly larger than on initial C T scan. Figure 3. Computed tomography scan after intraoperative injection of metrizamide into the fourth ventricle. (A) Metrizamide surrounds the cervicomedullary area. (B) Metrizamide in fourth ventricle (note layering). Slight contrast is visible in the pontocerebellar cisterns. Contrast not present in temporal horns. (C) Metrizamide in third and lateral ventricles.
70
Surg Neurol 1986;26:67-71
Figure 4. Computed tomography (CT~ scan with contrast l 7 months after initial C T scan, (A) Size of fourth ventricle is similar to that in prerious C T scan. Pathologicalenhancement is seen in hypothalamic area. (B) Third ventricle is enlarged but smaller than on 36th-day C T scan. Pathological enhancement adjacent to foramen on Monro. (C) Shunt in small right lateral ventricle. Enlargement of entire left latera/ ventricle.
the existing shunt proximal to the valve. After the procedure the patient's confusion partially resolved. On the third postoperative day, she complained suddenly of shortness of breath and progressed rapidly to cardiorespiratory arrest. Resuscitation attempts were unsuccessful. General autopsy demonstrated widespread granulomatous disease with severe involvement of the lungs, liver, spleen, and lymph nodes; the cardiac conduction system was uninvolved. There was no evidence of pulmonary thromboembolism. The ventriculoperitoneal shunt was patent. Examination of the brain and spinal cord disclosed severe involvement of the basal meninges, hypothalamus, pituitary stalk and gland, cranial and spinal nerves, choroid plexuses, and ventricular surfaces with noncaseating granulomas in various stages of evolution (Figure 5). At the level of the fourth ventricle, the ependymal involvement penetrated the medulla oblongata to a depth of 1 cm (Figure 6). The foramina of Luschka and Magendie were obliterated. In the spinal cord, the lumbosacral segments were more markedly involved.
Discussion Previously reported mass lesions in neurosarcoidosis have been parenchymal tumors, excrescences from basal
Schlitt et al
meninges and choroid plexuses, or meningocerebral infiltrates [3,4,6,7,10,11]. A loculated portion of the ventricular system presenting as a mass lesion has been reported twice [1,2]. Several reports [4,6,10] have suggested steroids as the mainstay of therapy in neurosarcoid mass lesions. Steroids were used only in an adjunctive manner in the treatment of the loculated ventricle in the present case. Because no egress from the trapped ventricle existed prior to operation, steroid medication would not have been expected to effect resolution. Computed tomography scans demonstrated adequate control of this aspect of the patient's disease, as manifested by lack of mass effect of the temporal horn of the lateral ventricle. Hydrocephalus complicating neurosarcoidosis is becoming more frequently recognized with the availability of CT scanning [1,2]. Prior to CT, hydrocephalus was not always recognized as the cause of intracranial hypertension, and patients often became blind or bedbound, or died after exploration of the posterior fossa [4]. More recently, several patients have been shunted; long-term outcome has varied from death to good neurological recoveries [4]. Hydrocephalus in neurosarcoidosis can be caused by granulomas in the third ventricle, aqueduct of Sylvius, or fourth ventricular outlets or by obstruction of the basal meninges [3,7,11]. Pathological evidence of defects in CSF reabsorption at the arachn0id villi has not been described. The cause of hydrocephalus in this case was obstruction of the outlets of the fourth ventricle and obliteration of the basal cisterns, as metrizamide injected into the fourth ventricle proved communication of all chambers at the time of the original placement of a shunt. Later, progressive
Neurosarcoidosis and Hydrocephalus
Surg Neurol 1986;26:67-71
71
Figure 6. Masses of granulomatous tissue filling the caudal angle of the fourth ventricle and infiltrating its floor.
Figure 5. Multiple confluent granulomas replacing most of the normal Hssue in the floor of the third ventricle. The asymmetry of the lateral ventricles is in part due to the adhesions of the ventricular walls produced by the granulomas.
granulomatous ependymitis isolated the right lateral ventricle from the remainder of the ventricular system, necessitating a left lateral ventricular catheter. The patient's compliance with the prescribed steroid therapy was uncertain and may have adversely influenced her outcome. Even so, histologic examination proved granulomas at various stages of evolution, suggesting that steroid administration could not have been expected to effect complete resolution of her neurosarcoidosis. The cause of death is uncertain but it is suspected that invasion of the medulla oblongata by the granu!omatous process may have produced some disturbances in the function of respiratory centers in the floor of the fourth ventricle, in contrast, despite virtually complete replacement of the hypothalamus and pituitary stalk and gland with sarcoid granulomas, the patient manifested no clinical signs of endocrine deficiency. Sarcoidosis is a chronic disease with protean systemic and neurologic manifestations. Lifelong observation with detailed neurologic examinations is required. Even so, data suggest neuromuscular involvement in more patients than is suspected clinically [4]. A high index of
suspicion of neurologic disease is therefore warranted regarding these patients, Serial CT scans should be performed, if intracranial pathology is suspected. Several different mass lesions and hydrocephalus can occur in neurosarc0idosis, necessitating surgical and medical management for optimal patient outcome. References 1. Bahr AL, Krumholz A, Kristt D, Hodges FJ III. Neuroradiological manifestations of intracranial sarcoidosis. Neuroradiology 1978;127:713-7. 2. Brooks BS, Gammal TE, Hungerford GD, Acker J, Trevor RP, Russell W. Radiologic evaluation of neurosarcoidosis: role of computed tomography. AJNR 1982;3:513-21. 3. Colover J. Sarcoidosis with involvement of the nervous system. Brain 1948;71:451-75. 4. Deianey P. Neurologic manifestations in sarcoidosis. Review of the literature, with a report of twenty-three cases. Ann Intern Med 1977;87:336-45. 5. Grizzanti JN, Knapp AB, Schecter AJ, Williams MH Jr. Treatment of sarcoid meningitis with radiotherapy. Am J Med 1982;73:605-8. 6. Healton EB, Zito G, Chauhan P, BrustJCM. Intracranial subdural sarcoid granuloma. Case report. J Neurosurg 1982;56:728-31. 7. Herring AB, Urich H. Sarcoidosis of the central nervous system. J Neurol Sci 1969;9:405-22. 8. James DG. Sarcoidosis. In: Hoeprich PD, ed. Infectious diseases. 2nd ed. Hagerstown, Maryland: Harper & Row, 1977:1203-9. 9. Neville E, Walker AN, James DG. Prognostic factors predicting outcome of sarcoidosis: an analysis of 818 cases. Q J Med 1983;52:525-33. 10. Powers WJ, Miller EM. Sarcoidosismimickingglioma: case report and review of intracranial sarcoid mass lesions. Neurology 1981;31:907-10. 11. Urich H. Neurological manifestations of sarcoidosis. Practitioner 1969;202:632-6.