Background Large cerebral infarction is a rare complication of neurocysticercosis. Endarteritis by inflammation of the leptomeninges is known to be its cause.
Case Description A 59-year-old man with known neurocysticercosis developed a large cerebral infarction during praziquantel therapy. A follow-up MRI obtained immediately after his cerebral infarction demonstrated notable decrease in the size of the cysts and more prominent enhancement around the peripheral margins of the cysts and the major vessels in comparison with the initial MRI. Cerebral angiography disclosed occlusions and narrowing of both internal carotid arteries at the supraclinoid portions, where multiple cysts were found on the MRI.
Conclusions Findings in our patient strongly suggest that a secondary inflammation reaction caused by the destruction of the cysts might have enhanced the process of endarteritis. The possible deleterious effects of praziquantel therapy should be considered in the treatment of patients with subarachnoid cysticerci.
Ischemic stroke is recognized as a complication of NCC.1 2 3 4 5 6 7 8 9 10 11 12 However, large infarctions have rarely been reported.4 5 6 7 8 9 10 Pathological studies indicate that endarteritis by inflammation of the leptomeninges and subsequent occlusion of the vessels are the cause of large cerebral infarction.6 11 Similarly, it has been proposed that a secondary inflammatory reaction in response to the destruction of the cysts by praziquantel therapy may enhance the process of endarteritis.2 5
A 59-year-old right-handed man with known NCC developed abrupt aphasia and right hemiplegia during the second course of praziquantel therapy. He smoked half a pack of cigarettes per day for 30 years and habitually ate undercooked pork. Two months before admission, he was evaluated at the Department of Neurosurgery because of progressive short-steppage gait disturbance. A brain MRI (Signa, 1.5 T) showed multiple cystic lesions of various stages, midbrain deformity from compression by these cysts, communicating hydrocephalus, and enhancements on the basal subarachnoid space (Fig 1A and 1B⇓⇓). His CSF contained WBC 13/mm3 (54% mononuclear, 41% eosinophilic, 3% basophilic, and 2% polymorphonuclear), RBC 83/mm3, 84 mg/dL protein, and 75 mg/dL sugar. CSF pressure was 210 mm H2O. Serological test results for anticysticercus IgG antibodies by micro-ELISAs were positive both in the CSF and the serum (absorbance value, 0.98 in the CSF, 1.02 in the serum; cutoff value, 0.18). After diagnosis of NCC and communicating hydrocephalus was determined, praziquantel (50 mg/kg) was administrated for 14 days, and a ventricular-peritoneal shunt operation was performed. Over the ensuing weeks, the patient's gait disturbance improved.
After a 2-month follow-up, the patient was readmitted for a second course of praziquantel therapy. His CSF contained WBC 84/mm3 (82% mononuclear, 17% eosinophilic, and 1% polymorphonuclear) and 192 mg/dL protein. After administration of oral praziquantel (50 mg/kg) and prednisolone (30 mg/d) for 2 days, he complained of several transient episodes of a tingling sensation and weakness in his right arm lasting for several minutes. A few hours later, he developed aphasia and right hemiplegia.
His blood pressure was 120/80, and his pulse was regular at 72 beats per minute. There were neither audible carotid bruits nor cardiac murmurs. Neurological examination revealed a drowsy mental status with global aphasia and severe right hemiplegia. His eyeballs were conjugately deviated to the left side. Stretch reflexes were increased in his right arm and leg.
Laboratory data revealed complete blood count, urinalysis, and SMA-12 findings to be normal. Erythrocyte sedimentation rate was 64 mm/h (corrected, 34). An immediate follow-up MRI showed a notable decrease in the size of the cysts and more prominent enhancement around the peripheral margins of the cysts and leptomeninges (Fig 1C and 1D⇑⇑). A brain CT scan taken 1 day later showed focal low-density lesions in the distribution of the left MCA territory (Fig 2⇓). The patient was transferred to the Department of Neurology because of his cerebral infarction.
Although we performed additional laboratory studies (including levels of triglyceride, HDL, rheumatoid factor, antinuclear antibodies, antithrombin III, protein C, and protein S and VDRL and echocardiography), we failed to find risk factors for stroke. The angiography showed complete occlusions of the right MCA and left ICA at the supraclinoid portion (Fig 3⇓). Intravenous dexamethasone (20 mg daily) and heparin were added to his praziquantel therapy. Two days after the onset of his right hemiplegia, however, the patient developed weakness in his left extremities and became abulic. A follow-up routine CSF study and ELISA tests performed 1 month later showed slight improvement. However, his clinical status had not improved by a 3-month follow-up examination.
NCC is a rare cause of large cerebral infarction.4 5 6 7 8 9 10 It is documented that subarachnoid cysticerci produce abnormal thickening of the basal leptomeninges, secondary to an inflammatory exudate.1 3 Blood vessels arising from the circle of Willis are usually trapped within this exudate and invaded by inflammatory cells. The end result of this process is endarteritis and subsequent infarction.1 3 6
Del Brutto3 suggests that the diagnosis of cysticercosis-induced cerebral infarction should be established only in patients who have no other risk factors for stroke and who show CT evidence of a meningeal cyst adjacent to the infarction or CSF findings compatible with active arachnoiditis. Findings in our patient strongly suggested that NCC was the primary cause of a cerebral infarction. Extensive medical workup failed to reveal risk factors for stroke other than smoking in our patient. Moreover, cerebral angiography disclosed occlusions of the ICA at the level where multiple cysts were found in the MRI. Pleocytic CSF findings and postcontrast MRI findings further supported active arachnoiditis around the sites of the occlusions.
To the best of our knowledge, 13 angiographically documented patients with compromise of large intracranial arteries have been reported.4 5 6 7 8 9 10 None of them, however, showed such extensive bilateral ICA occlusion as our patient. It has been described that transient neurological dysfunctions may develop either by a vascular compromise or parenchymal brain cysts.3 Our patient had several episodes of transient tingling and weakness in his right upper extremity and a subsequent left MCA infarction. Clinical and angiographic correlation clearly indicated that the occlusion of the left ICA was the cause.
Unfortunately, our patient developed his stroke during praziquantel therapy. He had taken praziquantel 2 months before the infarction and was taking it when he developed his catastrophic attack. There have been few reported cases in which cerebral infarction developed during antihelminthic treatment.2 5 Moreover, none have provided clear evidence that a secondary inflammation reaction caused by the destruction of the cysts enhanced the process of endarteritis, as in our patient. Additionally, a follow-up MRI demonstrated a notable decrease in the size of the cysts, as well as more prominent enhancement around the peripheral margins of the cysts and signal voids of the major vessels. These findings strongly suggest that a secondary inflammatory reaction caused by the destruction of the cysts might have enhanced the course of endarteritis. In addition to this acute reaction, a recent immunologic study also suggests that the long-term neuroimmunologic effect of the therapy lasts a minimum of 6 months.13 These findings imply that the process of endarteritis in our patient might have been enhanced by an acute inflammatory reaction, by delayed-type hypersensitivity, or by both.
Del Brutto et al14 suggest that the simultaneous coadministration of corticosteroids and albendazole is mandatory to avoid the cerebral infarction subsequent to angiitis caused by an acute inflammatory reaction. Although our patient received a simultaneous course of corticosteroids, this did not prevent his large cerebral infarction. However, findings in our patient do not necessarily mean that the coadministration of corticosteroids is ineffective, not only because the doses of corticosteroids used in our patient may have been insufficient but also because they may have been given too late to prevent an acute inflammatory reaction.
In conclusion, we suggest that the known potential deleterious effect of praziquantel therapy on the process of endarteritis should be considered when treating patients with subarachnoid cysticerci. Our findings add to the growing evidence that the early start of high doses of corticosteroids may be mandatory in patients with subarachnoid cysticerci who are scheduled for use of antihelminthic drugs.
Selected Abbreviations and Acronyms
|ELISA||=||enzyme-linked immunosorbent assay|
|ICA||=||internal carotid artery|
|MCA||=||middle cerebral artery|
|RBC||=||red blood cells|
|WBC||=||white blood cells|
The authors wish to thank to Carole Cameron Shaw for her editorial assistance in English.
- Received June 3, 1996.
- Revision received September 16, 1996.
- Accepted September 16, 1996.
- Copyright © 1997 by American Heart Association
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