Journal of Neurological Sciences 154 (1998) 94–100 Uncommon presentations of neurocysticercosis a, a a b a I.M.S. Sawhney *, G. Singh , O.P. Lekhra , S.N. Mathuriya , P.S. Parihar , S. Prabhakar a a b Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India Received 27 December 1996; received in revised form 18 June 1997; accepted 27 June 1997 Abstract Neurocysticercosis commonly presents with seizures, raised intracranial tension and dementia. The unusual location of the cysts may result in uncommon manifestations mimicking a host of neurological disorders. Ten patients with neurocysticercosis with rare clinical presentations have been described in this series. These include dorsal midbrain syndrome, isolated bilateral ptosis, papillitis, cerebral hemorrhage, painful cervical radiculopathy, progressive swelling of arm, paraplegia due to intramedullary cyst, third ventricular cyst, dystonia and nominal aphasia masquerading as transient ischaemic attacks. The clinical details and possible mechanisms for these rare presentations are discussed.  1998 Elsevier Science B.V. Keywords: Neurocysticercosis; Uncommon manifestations; Mesencephalic syndrome; Cerebral hemorrhage; Papillitis; Dystonia; Nominal aphasia; Intramedullary cyst 1. Introduction Neurocysticercosis is a common parasitic infection of the central nervous system in the Indian subcontinent, central and south America, Spain and Eastern Europe (ILAE, 1994; Schantz et al., 1994; Tsang and Wilson, 1995). Its incidence is rising in the non-endemic areas because of rising rates of immigration from endemic countries (DelBrutto et al., 1988; Sorvillo et al., 1992; Shandera et al., 1994). Clinical manifestations depend upon cyst load, topographic location of cysts and the host’s immune response (DelBrutto et al., 1988). The disease has pleomorphic presentations. The common clinical features are seizures, raised intracranial tension and dementia (Sotelo et al., 1985; Wei et al., 1988). The uncommon clinical manifestations include pure motor hemiparesis, ataxic hemiparesis, homonymous hemianopia, Parinaud’s syndrome, cerebellopontine angle syndrome, Brown-Sequard syndrome and stroke (Kaene, 1982; McCormick, *Corresponding author. 0022-510X / 98 / $19.00  1998 Elsevier Science B.V. All rights reserved. PII S0022-510X( 97 )00206-2 1985; Sotelo et al., 1985; Sharma et al., 1987; Barinagarrementeria and DelBrutto, 1988, 1989). It is important to recognise the rare presentations of the disease as these can mimic a number of other neurological disorders. The lack of awareness of these uncommon presentations may lead to wrong diagnosis, especially in tropical countries where diagnostic facilities are limited because of a paucity of resources. We report some of the rare clinical manifestations. 2. Patients Ten cases of neurocysticercosis with rare clinical manifestations were admitted to the department of Neurology and Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India, in the last ten years. The diagnosis of cysticercosis was made by clinical course and radiological tests. Immunological tests for cysticercosis were positive in cases 4 and 10. Histo- Age (years)/sex 42/ male 4/ Female 7/ Female 10/ Male 13/ Male 45/ Female Case no. 1 2 3 4 5 6 Table 1 Case summaries of neurocysticercosis Gradually progressive swelling and pain over right arm Severe neck pain, headache, vomiting and neck rigidity Painful restriction of neck movements and bilateral papilloedema Developed seizures in later part of illness Plain x-ray right arm— multiple radiopaque lesions (Fig. 3a–b) Cervical spine x-rays —normal Cranial CT and myelo CT —normal CSF cells—normal, protein5 90 mg%, normal sugar MRI—multiple cysts Tonsillar hemiation CT scan on admission —normal Repeat CT—left parietal hematoma (Fig. 2) Four vessel angiography —normal MRI—multiple cysts MRI—multiple cysticerci Developed recurrent seizures, raised ICT and bilateral post papillaematous optic atrophy within two months of visual symptoms Headache, vomiting, right partial motor seizures for five days Developed right hemiparesis six weeks later CT scan—white matter oedema only CT scan—bilateral multiple ring-enhancing lesions with perifocal oedema One lesion in midbrain (Fig. 1) CT scan—multiple hypodense lesions left frontal, parietal and occipital regions Mild hydrocephalus Investigations Rapid diminution of vision and disc oedema in right eye (papillitis) for seven days Fully conscious, bilateral papilloedema bilateral isolated ptosis with intact pupillary reflexes and ocular movements Low grade fever and headache for two months drooping of eyelids for two weeks Fully conscious, bilateral papilloedema, skew deviation of eyes, upward gaze palsy, left hemiparesis, bilateral extensor plantars Left partial motor seizures for eight years Diplopia, gradual loss of vision and swaying to the left side for two months Clinical features Operation was done AVM riddled with clumps of cysts Complete recovery Steroids Praziquantel Anticonvulsants Antituberculosis therapy—no response No improvement Steroids Albendazole Anticonvulsants Steroids Albendazole Seizures and raised ICT controlled; vision—no recovery Steroids Almost complete recovery Anticonvulsants Steroids Ventriculo peritoneal shunt—no relief Right partial frontal lobectomy with enucleation of cysts— had partial relief Therapy and outcome AVM riddled with cysts Tonsillar herniation Cervical radiculopathy Haemorrhagic stroke Papillitis Mesencephalic syndrome Isolated bilateral ptosis Mesencephalic syndrome, upward gaze palsy, skew deviation Uncommon features Muscle Posterior fossa Left parietal lobe Optic nerve Midbrain Midbrain Localisation I.M.S. Sawhney et al. / Journal of Neurological Sciences 154 (1998) 94 – 100 95 96 Table 1. Continued Age (years)/sex Clinical features Investigations Therapy and outcome Uncommon features Localisation 7 28/ Male Pain in upper dorsal region followed by paraesthesia in lower limbs Myelo CT—intramedullary lesion at D 1 MRI—spine neurocysticercosis cyst (Fig. 4) CSF: 40 cells/mm 3 Lymphocytes: 50% Polymorphs: 50% Protein: 80 mg% Sugar: 30 mg% Operation—whitish cyst (cysticercus) 1 cm in size Compressive myelopathy Intramedullary cyst 2 Third ventricular cyst Developed complete paraplegia with D 2 sensory level and bladder involvement 8 16/ Female Headache and progressive diminution of vision for two years Raised ICT, diplopia for twenty days 9 21/ Male Left side frontal headaches associated with occasional vomiting for two years Complex partial seizures for two years Episodic dystonic posturing of right upper and lower limbs lasting from a few minutes to hours 10 45/ Female Hypertension for five years Three episodes of transient aphasia lasting for 10–15 min Improved—grade 4 muscle power and full control of bladder in six months Skull X-ray erosion of dorsum sellae CT—dilated lateral ventricles isodense mass in anterior third ventricle Conray ventriculogram— cyst in anterior third ventricle (Fig. 5) Operation—single cyst of NCC removed EEG: generalised epileptiform discharges CT: hypodense cysts pars interna of globus pallidi, ringenhancing lesion in left corona radiata, bifrontal diffuse white matter oedema Steroids Anticonvulsants Improved Unilateral dystonia Temporal/ frontal lobe CT—ring-enhancing lesion with mild perifocal oedema over left Sylvian fissure Asymptomatic on anticonvulsant therapy Transient ischaemic attacks Left Sylvian fissure ICT, intracranial tension; AVM, arteriovenous malformation; NCC, neurocysticercosis. Symptoms relieved one week after surgery I.M.S. Sawhney et al. / Journal of Neurological Sciences 154 (1998) 94 – 100 Case no. I.M.S. Sawhney et al. / Journal of Neurological Sciences 154 (1998) 94 – 100 pathological confirmation was available in four cases. The cases are summarised in Table 1. 3. Discussion The uncommon presentations of neurocysticercosis in this series (Table 1) are as follows: 3.1. Mesencephalic syndrome The clinical features of this syndrome found in the cases studied include upward gaze palsy, convergence retraction nystagmus, eyelid retraction, pupillary abnormalities and skew deviation. The common underlying pathology is obstructive hydrocephalus (Kaene, 1982) due to a cyst in the third or fourth ventricle or granulomatous ependymitis secondary to degenerating intraventricular cyst. It can result from communicating hydrocephalus, which develops in 25% of cases of neurocysticercosis because of arachnoiditis or meningeal fibrosis (Lobato et al., 1981; Sotelo et al., 1985; Sotelo and Martin, 1987). The syndrome has also been seen because of cysticercoid arachnoiditis involving the mid brain. The arachnoid adhesions in the interpeduncular and prepontine cisterns lead to occlusion of paramedian and mesencephalic arteries, resulting in multiple areas of ischaemic necrosis in the mesencephalon. Finally, a parenchymal cyst located in the dorsal mid brain may be responsible for this syndrome (Fig. 1). This mechanism appears to be operative in case nos. 1 and 2. 97 3.2. Papillitis Papilledema resulting in optic atrophy is well known in cases of neurocysticercosis with chronic raised intracranial tension. However, papillitis as a presenting feature of neurocysticercosis has not been documented. The rapid loss of vision associated with disc oedema and the absence of symptoms of raised intracranial tension at the time of first presentation are consistent with the diagnosis of papillitis in case no. 3. She developed symptoms of raised intracranial tension and seizures two months later. 4. Stroke The association of cerebrovascular disease with stroke is well recognised (Sotelo et al., 1985; Barinagarrementeria and DelBrutto, 1988, 1989; Penring et al., 1992; DelBrutto, 1992; Monteiro et al., 1994; Levy et al., 1995). The incidence of stroke in neurocysticercosis varies from 2.0 to 11.8% (Sotelo et al., 1985; DelBrutto, 1992). Vascular occlusion can occur as a result of superficial cortical vessel thrombosis due to chronic meningitis (Grisolia and Wiederholt, 1982), vasculitis (Sotelo et al., 1985), lacunar infarcts due to endarteritis of small vessels (Barinagarrementeria and DelBrutto, 1988, 1989) and occlusion of the middle cerebral artery due to cysts or arachnoiditis (Penring et al., 1992). All the cases reported in literature so far presented with occlusive stroke. Case no. 4 is unique because of the presence of parenchymal hemorrhage (Fig. Fig. 1. Contrast-enhanced CT scan showing multiple ring lesions of neurocysticercosis. There is a ring-enhanced lesion in the mid brain (thick arrow) responsible for mesencephalic syndrome. The thin arrow shows the quadrigeminal plate cistern around the mid-brain. 98 I.M.S. Sawhney et al. / Journal of Neurological Sciences 154 (1998) 94 – 100 imaging (MRI) confirmed the diagnosis of multiple cysticerci in the posterior fossa and inferior displacement of the tonsils. Tonsillar herniation was the cause of neck pain in this case. Nuchal pain and rigidity can occur in neurocysticercosis because of raised intracranial tension or meningitis. The clinical differentiation of these two conditions can be difficult because of the overlapping symptoms. The intracranial hypertension due to neurocysticercosis can prove to be life threatening. Chronic arachnoiditis is a frequent complication of cisternal cysticercosis and is associated with a prolonged course, multiple relapses and a high case-fatality rate (Joubert, 1993). 5. Intramedullary spinal cyst Fig. 2. Non-contrast CT scan showing a left parietal hematoma. 2). The possible mechanism of parenchymal hemorrhage is rupture of mycotic aneurysm (Grisolia and Wiederholt, 1982), erosion of a degenerating cyst in the wall of a small vessel or a vasculitic hemorrhage (Sotelo et al., 1985). 4.1. Painful cervical radiculopathy Cervical radiculopathy is a rare manifestation of neurocysticercosis. Case no. 5 presented with severe nuchal pain. As cranial CT was normal, a myelo CT was performed to rule out a high cervical compressive lesion and it did not reveal any abnormality. Magnetic resonance In a large series of neurocysticercosis, 1–3.2% of cases were of spinal cysticercosis (Sotelo et al., 1985). Both intradural extramedullary and intramedullary (Queiroz et al., 1975; Garza-Mercado, 1976; Akiguchi et al., 1979; Kim and Weinberg, 1985; Savolardo et al., 1986) forms have been reported. Intramedullary cysticercosis is much less common than the extramedullary leptomeningeal disease. Sharma et al. (1987) reviewed the published literature of intramedullary spinal cysticercosis and found 32 published cases. Fifty six per cent of intramedullary cysts are located in the thoracic cord (Fig. 4). This predilection of the cysts for the thoracic spinal cord is linked to the regional spinal cord blood flow, which is highest in the thoracic cord. The presence of rapid spontaneous worsening because of the inflammatory re- Fig. 3. (a) Radiograph of the limb showing soft tissue swelling with calcification. (b) Pathology specimen of the same showing multiple cysticerci. I.M.S. Sawhney et al. / Journal of Neurological Sciences 154 (1998) 94 – 100 99 5.2. Dystonia Unilateral dystonic posturing is an unusual feature of epilepsy and has been reported in complex partial seizures of temporal origin (Kotgal et al., 1989). Dystonia is contralateral to the epileptic focus and is probably due to the spread of ictal discharge to subcortical structures, namely the basal ganglia. The dystonia in case no. 9 was epileptic in nature, as the patient had typical complex partial seizures and electroencephalogram (EEG) abnormality. However, some of the attacks lasted for hours, mimicking symptomatic dystonia. 5.3. Transient ischaemic attacks Fig. 4. MRI with T2 weighted image showing an intramedullary cyst in the dorsal spinal cord. action to the cyst, associated systemic or cerebral cysticercosis, cerebrospinal fluid eosinophilia or a positive cerebrospinal fluid complement fixation test are features that may arouse suspicion that cysticercosis is the underlying pathology in compressive myelopathies. 5.1. Ventricular cysts Intraventricular cysts are uncommon cysts that usually are larger than parenchymal cysts and may be free-floating or attached to the ventricular wall (Zee et al., 1984). The common site for these cysts is the fourth ventricle (Zee et al., 1993). Cysts in the third ventricle, as seen in case no. 8 (Fig. 5), are rare (Salazar et al., 1983). These cysts become symptomatic by blocking the aqueduct of Sylvius, resulting in obstructive hydrocephalus and raised intracranial tension. Fig. 5. Conray ventriculogram showing cyst (arrows) in the anterior third ventricle. Case No. 10 was diagnosed to have transient ischaemic attacks because of recurrent transient neurological deficits and hypertension. The diagnosis of neurocysticercosis was established after the CT scan. The recurrent transient neurological deficit was due to partial seizures. It is well known that partial seizures can mimic transient ischaemic attacks. The pleomorphic clinical presentation of neurocysticercosis can mimic a number of neurological syndromes. When a patient presents with rare clinical features, the diagnosis may be missed, especially in developing countries where investigative facilities are limited. 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