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Increased intracranial pressure and hydrocephalus in a teenager
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ILLUSTRATED CONTINUING MEDICAL EDUCATl0N
Increased intracranial pressure and hydrocephalus in a teenager Clin Microbiol Infect 1999; 5: 164-165
Nicolas Eoillet
Lkonard Gianadda2, Christophe Duc', and Jacques De Preux
'Infectious Diseases and Pathology Divisions, Central Institute of the Valais Hospitals, 2Division of Neurosurgery, Regional Hospital, Sion, Switzerland
Figure 1 Ventricular neurocysticercosis in an adopted teenager who left Bolivia 14 years ago.
revealed a solitary 1.5 cm round cystic lesion in the left lateral ventricle that was not visible on the CT. It was attached to the choroid plexus and obstructed CSF circulation (see figure). Because of clinical deterioration, the patient rapidly underwent surgical ventriculostomy for drainage and removal of the cyst. Histology established the diagnosis.
An otherwise healthy 15-year-old male was transferred from another hospital to our institution on March 28, 1997, because of the rapid onset of signs compatible with increased intracranial pressure (ICP) and the presence of hydrocephalus on a computerized tomography (CT) scan of the head. He had been adopted from Bolivia in 1983 and had not left Western Europe nor had close contacts with individuals from developing countries during this time period. Two months earlier, he had suffered from spontaneously resolving headache, fever, nausea, and vomiting that had been attributed to a viral meningitis (sterile CSF with transient lymphocytic pleiocytosis and elevated proteins). O n admission to our hospital, magnetic resonance imaging (MRI)
QUESTIONS
1. 2. 3. 4. 164
What What What What
is the diagnosis? other methods of diagnosis are available? are the usual clinical features of this disease? are the possibilities for treatment?
Troillet et al: Increased intracranial pressure and hydrocephalus in a teenager
Stained sections of the cyst revealed a scolex consistent with Cysticercus cellulosae. The diagnosis is ventricular neurocysticercosis. Despite a low sensitivity in cases with single parenchymatous cysts, serodiagnosis by immunoblot may be positive, both in serum and CSF, in more than 80% of patients with meningeal irritation and intraventricular cysts, thus providing a helpful diagnostic tool in conjunction with MRI. Though intraventricular cysts may be present in up to one third of patients with active neurocysticercosis, they coexist most often with parenchymatous cysts which may cause seizures, the most common symptom at presentation (70%). Headache (8%) and symptoms of hydrocephalus (5%) are more rare. As stated by Cuetter et al., the current treatment standards for ventricular neurocysticercosis imply either a ventriculoperitoneal shunt or a temporary ventriculostomy when increased ICP due to hydrocephalus occurs. Surgical removal of the cysts must be performed if they are big, obstruct CSF flow, or complicate shunt functioning. In patients with intraventricular cysts without hydrocephalus, or with only slight dilatation of the ventricles, these authors favor first a trial of albendazole therapy alone, under close supervision (15 mg/kg/day given for two 15-day treatment courses, one month apart, initially with steroids). They otherwise combine this therapy with ventriculoperitoneal shunt. Three of four patients, however, who underwent a diversionary procedure in their series required repeated procedures because of obstruction of the shunt, a fact that could justify early surgical removal of the cyst when feasible.
dreadful illness that should be suspected in immigrants from endemic areas in the world presenting with neurological signs or symptoms, even many years after they left their country. Indeed, the absence of contact with potential carriers of 'T: solium since his adoption, and the absence of proglottides or eggs on repeated stool analyses, strongly suggest that this patient acquired cysticercus at least 14 years earlier, before coming to Switzerland. Clinical manifestations probably occurred at the third of the four stages of neurocysticercosis, the colloid cyst stage that usually takes place 2 to 10 years after infection. This stage is characterized by the leakage of antigens through the cyst's deteriorating capsule, before it dies and calcifies. In case of ventricular involvement, the resulting inflammatory response may cause ventriculitis and a localized reaction that fixes the cyst to the ventricular wall with thickening and secondary blockade of the CSF circulation. Ventricular neurocysticercosis alone, without parenchymatous cysts, is rare and can be difficult to diagnose. Unlike the classical form of the disease, it usually does not present with seizures but rather causes increased ICP or less overt signs or symptoms including the aseptic meningitis with which our patient initially presented. Moreover, as illustrated also by this report, ventricular cysts may not be seen on CT. Specific antibodies were found in our patient's serum and CSF by ELISA and immunoblot (Tropeninstitut, Basel, Switzerland). The patient was discharged on June 14, after a short course of dexamethasone and initiation of albendazole for one month. Seven months later, his neurological signs and symptoms has resolved, the MRI showed no residual hydrocephalus or cyst, and serum antibodies had disappeared. N. Troillet Division of Infectious Diseases, Central Institute of the Valais Hospitals, 86, Ave. Grand-Champsec, CH-1950 Sion, Switzerland
DISCUSSION
Neurocysticercosis consists of infection of the central nervous system with the larval stage of Taenia solium, Cysticercus cellulosae. Whereas human intestinal infection with the tapeworm results from ingestion of undercooked pork containing viable cysticerci, cysticercosis is acquired by ingestion of 'T: solium eggs from food, water or hands contaminated with feces of human carriers. Self-infection is possible via the fecal-oral route. The estimated median incubation period of this disease is 3.5 years. Neurocysticercosis has been virtudy eliminated from industrialized nations, but it still affects millions of people in the developing countries of Latin America, Asia, and Africa. Paralleling the increase in immigration of individuals from endemic areas, an increase in the prevalence of neurocysticercosis has been reported in the United States. This case presents several instructive features. It demonstrates first that neurocysticercosis may be a
165
Tel: +41 27 203 8151 Fax: +41 27 203 2975 E-mail: [email protected]
Further reading 1. Botero D, Tanowitz HB, Weiss LM, Wittner M. Taeniasis and cysticercosis. Infect Dis Clin N Am 1993; 7: 683-97. 2. Davis LE. Neurocysticercosis: pathophysiology, diagnosis, and management. Infect Dis Clin Pract 1997; 6: 358-65. 3. Shandera WX, White AC, Chen JC, Diaz P, Armstrong R. Neurocysticercosis in Houston, Texas. A report of 112 cases. Medicine 1994; 73: 37-52. 4. Cuetter AC, Garcia-Bobaddla J, Guerra LG, Martinez FM, Kaim B. Neurocysticercosis: focus on intraventricular disease. Clin Infect Dis 1997; 24: 157-64. 5. Wilson M , Bryan RT, Fried JA, et al. Clinical evaluation of the cysticercosis enzyme-linked immunoelectrotransfer blot in patients with neurocysticercosis.J Infect Dis 1992; 164: 1007-9.
ILLUSTRATED CONTINUING MEDICAL EDUCATl0N
Increased intracranial pressure and hydrocephalus in a teenager Clin Microbiol Infect 1999; 5: 164-165
Nicolas Eoillet
Lkonard Gianadda2, Christophe Duc', and Jacques De Preux
'Infectious Diseases and Pathology Divisions, Central Institute of the Valais Hospitals, 2Division of Neurosurgery, Regional Hospital, Sion, Switzerland
Figure 1 Ventricular neurocysticercosis in an adopted teenager who left Bolivia 14 years ago.
revealed a solitary 1.5 cm round cystic lesion in the left lateral ventricle that was not visible on the CT. It was attached to the choroid plexus and obstructed CSF circulation (see figure). Because of clinical deterioration, the patient rapidly underwent surgical ventriculostomy for drainage and removal of the cyst. Histology established the diagnosis.
An otherwise healthy 15-year-old male was transferred from another hospital to our institution on March 28, 1997, because of the rapid onset of signs compatible with increased intracranial pressure (ICP) and the presence of hydrocephalus on a computerized tomography (CT) scan of the head. He had been adopted from Bolivia in 1983 and had not left Western Europe nor had close contacts with individuals from developing countries during this time period. Two months earlier, he had suffered from spontaneously resolving headache, fever, nausea, and vomiting that had been attributed to a viral meningitis (sterile CSF with transient lymphocytic pleiocytosis and elevated proteins). O n admission to our hospital, magnetic resonance imaging (MRI)
QUESTIONS
1. 2. 3. 4. 164
What What What What
is the diagnosis? other methods of diagnosis are available? are the usual clinical features of this disease? are the possibilities for treatment?
Troillet et al: Increased intracranial pressure and hydrocephalus in a teenager
Stained sections of the cyst revealed a scolex consistent with Cysticercus cellulosae. The diagnosis is ventricular neurocysticercosis. Despite a low sensitivity in cases with single parenchymatous cysts, serodiagnosis by immunoblot may be positive, both in serum and CSF, in more than 80% of patients with meningeal irritation and intraventricular cysts, thus providing a helpful diagnostic tool in conjunction with MRI. Though intraventricular cysts may be present in up to one third of patients with active neurocysticercosis, they coexist most often with parenchymatous cysts which may cause seizures, the most common symptom at presentation (70%). Headache (8%) and symptoms of hydrocephalus (5%) are more rare. As stated by Cuetter et al., the current treatment standards for ventricular neurocysticercosis imply either a ventriculoperitoneal shunt or a temporary ventriculostomy when increased ICP due to hydrocephalus occurs. Surgical removal of the cysts must be performed if they are big, obstruct CSF flow, or complicate shunt functioning. In patients with intraventricular cysts without hydrocephalus, or with only slight dilatation of the ventricles, these authors favor first a trial of albendazole therapy alone, under close supervision (15 mg/kg/day given for two 15-day treatment courses, one month apart, initially with steroids). They otherwise combine this therapy with ventriculoperitoneal shunt. Three of four patients, however, who underwent a diversionary procedure in their series required repeated procedures because of obstruction of the shunt, a fact that could justify early surgical removal of the cyst when feasible.
dreadful illness that should be suspected in immigrants from endemic areas in the world presenting with neurological signs or symptoms, even many years after they left their country. Indeed, the absence of contact with potential carriers of 'T: solium since his adoption, and the absence of proglottides or eggs on repeated stool analyses, strongly suggest that this patient acquired cysticercus at least 14 years earlier, before coming to Switzerland. Clinical manifestations probably occurred at the third of the four stages of neurocysticercosis, the colloid cyst stage that usually takes place 2 to 10 years after infection. This stage is characterized by the leakage of antigens through the cyst's deteriorating capsule, before it dies and calcifies. In case of ventricular involvement, the resulting inflammatory response may cause ventriculitis and a localized reaction that fixes the cyst to the ventricular wall with thickening and secondary blockade of the CSF circulation. Ventricular neurocysticercosis alone, without parenchymatous cysts, is rare and can be difficult to diagnose. Unlike the classical form of the disease, it usually does not present with seizures but rather causes increased ICP or less overt signs or symptoms including the aseptic meningitis with which our patient initially presented. Moreover, as illustrated also by this report, ventricular cysts may not be seen on CT. Specific antibodies were found in our patient's serum and CSF by ELISA and immunoblot (Tropeninstitut, Basel, Switzerland). The patient was discharged on June 14, after a short course of dexamethasone and initiation of albendazole for one month. Seven months later, his neurological signs and symptoms has resolved, the MRI showed no residual hydrocephalus or cyst, and serum antibodies had disappeared. N. Troillet Division of Infectious Diseases, Central Institute of the Valais Hospitals, 86, Ave. Grand-Champsec, CH-1950 Sion, Switzerland
DISCUSSION
Neurocysticercosis consists of infection of the central nervous system with the larval stage of Taenia solium, Cysticercus cellulosae. Whereas human intestinal infection with the tapeworm results from ingestion of undercooked pork containing viable cysticerci, cysticercosis is acquired by ingestion of 'T: solium eggs from food, water or hands contaminated with feces of human carriers. Self-infection is possible via the fecal-oral route. The estimated median incubation period of this disease is 3.5 years. Neurocysticercosis has been virtudy eliminated from industrialized nations, but it still affects millions of people in the developing countries of Latin America, Asia, and Africa. Paralleling the increase in immigration of individuals from endemic areas, an increase in the prevalence of neurocysticercosis has been reported in the United States. This case presents several instructive features. It demonstrates first that neurocysticercosis may be a
165
Tel: +41 27 203 8151 Fax: +41 27 203 2975 E-mail: [email protected]
Further reading 1. Botero D, Tanowitz HB, Weiss LM, Wittner M. Taeniasis and cysticercosis. Infect Dis Clin N Am 1993; 7: 683-97. 2. Davis LE. Neurocysticercosis: pathophysiology, diagnosis, and management. Infect Dis Clin Pract 1997; 6: 358-65. 3. Shandera WX, White AC, Chen JC, Diaz P, Armstrong R. Neurocysticercosis in Houston, Texas. A report of 112 cases. Medicine 1994; 73: 37-52. 4. Cuetter AC, Garcia-Bobaddla J, Guerra LG, Martinez FM, Kaim B. Neurocysticercosis: focus on intraventricular disease. Clin Infect Dis 1997; 24: 157-64. 5. Wilson M , Bryan RT, Fried JA, et al. Clinical evaluation of the cysticercosis enzyme-linked immunoelectrotransfer blot in patients with neurocysticercosis.J Infect Dis 1992; 164: 1007-9.