|Year : 2001 | Volume
| Issue : 2 | Page : 118-120
Multiple cranial nerve palsy in an HIV-positive patient
Satya Kama1, Jyotirmay Biswas1, N Kumarasamy2, Praveen Sharma2
1 MD. Medical Research Foundation Chennai, India
2 MD. YRG Centre for AIDS Research and Education Chennai, India
MD. Medical Research Foundation Chennai, India
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kama S, Biswas J, Kumarasamy N, Sharma P. Multiple cranial nerve palsy in an HIV-positive patient. Indian J Ophthalmol 2001;49:118-20
|How to cite this URL:|
Kama S, Biswas J, Kumarasamy N, Sharma P. Multiple cranial nerve palsy in an HIV-positive patient. Indian J Ophthalmol [serial online] 2001 [cited 2020 Apr 8];49:118-20. Available from: http://www.ijo.in/text.asp?2001/49/2/118/22647
We report the case of a 32 -year- old HIV-positive Indian male who presented with sixth nerve (bilateral), ninth, tenth and twelfth nerve palsies; cerebellar and posterior column involvement. CT scan showed gyriform enhancement in the right occipital lobe and nodular leptomeningeal enhancement in the left frontal lobe. Cytomegalovirus serology was positive and the patient was treated as presumed CMV. HIV can present with multiple cranial neuropathy and varied neurological involvement.
The first two cases of ocular lesions in AIDS were reported by us in 1995. Since then we have reported ocular lesions in 100 Human Immunodeficiency Virus (HIV) positive patients. In that series only one patient had a gaze palsy due to a glioma of the central nervous system. Neuro-ophthalmic manifestations are about 8% of the ocular lesions in AIDS patients. Neurological complications occur in approximately 40% of patients with AIDS, about 10-20% of them are initially examined because of neurological complaints.[2-4] Focal or diffuse neurologic dysfunction may occur any time during the clinical course and may affect the central or peripheral nervous system. Ocular cranial nerve palsies occur in 4% of patients with ocular involvement with AIDS. Palsy of the third, fourth, sixth and seventh cranial nerves in AIDS have been reported and may be bilateral or combined. A majority of these cases were due to focal brainstem toxoplasmic lesions. Others were due to cryptococcosis, varicella zoster, cytomegalovirus (CMV), progressive multifocal leucoencephalopathy, central nervous system and orbital lymphoma and cavernous and orbital apex eosinophilic granuloma. Hamed et al reported ocular motility manifestations of focal brainstem dysfunction as the initial clinical features in three patients with HIV infection. Cranial nerve palsies can be the earliest manifestation in a patient with AIDS.
We present a case of multiple cranial nerve palsy in an HIV-positive patient.
| Case history|| |
A 32-year-old Indian male gave a history of blurred vision in both eyes for a duration of two months. He complained of giddiness, headache and nausea. He also gave a history of weakness of limbs and slurred speech. He was detected as HIV positive 15 months ago and had been treated for pulmonary tuberculosis, oropharyngeal candidiasis and herpes zoster.
On examination his visual acuity was 6/6, N6 in both the eyes. There was restriction of abduction in both the eyes [Figure - 1]. Pupils were brisk in both the eyes. As he was very disoriented, he could not cooperate for confrontation fields. Slitlamp and fundus examination revealed no abnormality. Intraocular pressure (IOP) was normal in both the eyes. Systemic examination revealed oral candidiasis. An earlier neurological examination had showed cerebellar dysarthria, diminished left palatal movement and gag reflex, tongue deviated to the right, nasal regurgitation on swallowing, grossly impaired posterior column, flexor plantar reflex and ataxic gait. In short, there was involvement of the ninth, tenth and twelfth cranial nerves and of the cerebellar and posterior column.
Computed Tomography (CT) scan showed gyriform enhancement in the right occipital lobe and nodular leptomeningeal enhancement in the left frontal lobe [Figure - 2].
Sputum culture was positive for Streptococcus A cerebrospinal fluid (CSF) tap was done; bacterial, fungal and acid-fast bacillus direct smears were negative. Immunofluorescence staining for cytomegalovirus was negative. India Ink preparation for Cryptococcus was also negative. Culture for bacteria, fungus and acid-fast bacilli did not reveal any growth. The CSF sugar was 20 mg/dl, chloride was lOOmEq/litre and protein was 2.8 g/litre. Serum antibodies to cytomegalovirus, both IgG (459 EU) and IgM (166 EU), were highly positive. Serum antibodies to Herpes simplex virus and Toxoplasma were negative. TPHA and VDRL were non-reactive. The CD4 count was 244, CD8 count was 1421 and CD4: CD8 was 0.17.
The patient was presumed to have cytomegalovirus encephalitis and received intravenous Ganciclovir 5mg/kg body weight for 7 days. On re-evaluation 3 days later, his vision was 6/6, N6 in the right eye and counting fingers close to face in the left eye, though he was extremely disoriented. He died a week later.
| Discussion|| |
Cytomegalovirus encephalitis, a recognised opportunistic infection in HIV patients, is also known to cause cranial nerve palsies. In our opinion, this patient had CMV encephalitis in view of the high IgM and IgG antibody level to CMV. With clinical suspicion and positive antibodies to cytomegalovirus, the patient was treated with intravenous ganciclovir. However there was apparently no clinical response. Microbiological, histopathological and molecular biological study including PCR and viral culture, which could not be done due to financial constraints, would have established the aetiological diagnosis. However, it would be ideal to do the same on autopsy. CMV can cause cranial nerve palsies, optic neuritis, retrobulbar optic neuritis, slowed saccades, abnormal oculokinetic response and internuclear ophthalmoplegia.
The sudden loss of vision in our patient's left eye three days after presentation could be due to CMV retrobulbar optic neuritis. The imaging features of CMV encephalitis include periventricular white matter lesions and subependymal lesions, which were not seen in our patient. Up to 60-90% of patients with CMV encephalitis have retinitis. In our patient fundus examination could not be done after onset of blindness as the patient was toxic.
Increased intracranial pressure and mass effects by lesions such as lymphoma of the frontal lobe and contrictive arachnoiditis from cryptococcal meningitis can affect the ocular motor nerves at the base of the skull. Intravenous contrast enhancement of the lesions is usually a feature of toxoplasmic encephalitis, central nervous system lymphoma and cerebral infarct. The presence of gyriform enhancement in the right occipital lobe and nodular leptomeningeal enhancement in the left frontal lobe in our case could be indicative of a CNS lymphoma. Primary central nervous system non-Hodgkin's lymphoma is the most common neoplasm associated with AIDS. Generally patients present with confusion, lethargy and cognitive dysfunction. Focal neurological deficits and cranial neuropathy have been observed. CT scan demonstrates space-occupying lesions that enhance after intravenous contrast administration with a predilection for basal ganglia, thalami, corpus callosum, periventricular system and the vermis of the cerebellum. Multiple lesions have been noticed in 47% of the cases and in all cases at post-mortem examination. Such tumours respond favourably to radiotherapy although no significant effect on patient survival has been observed.
Cranial neuropathy in an HIV-infected patient may herald an otherwise occult systemic lymphoma. Repeated CSF examinations with immunocytochemical studies are mandated due to the difficulty in distinguishing reactive lymphocytes from lymphoma cells. Plain and gadolinium-enhanced Magnetic Resonance Imaging (MRI) of brain and spine (Tl and T2) need to be obtained. In our case MRI could not be done due to financial constraints. Chest and abdominal CT scans should be done, to detect the presence of any extra-CNS disease in such cases.
A negative serology for toxoplasma antibodies excluded toxoplasmosis. A negative India ink preparation for cryptococcosis excluded the same. Although progressive multifocal leucoencephalopathy can present as a cranial nerve palsy, the cranial lesions show a lack of contrast enhancement and a lack of mass effect on neuroimaging. The possibility of tuberculous aetiology cannot be ruled out.
HIV may result in a variety of neuro-ophthalmic manifestations, including papilledema, papillitis, optic atrophy, retrobulbar neuritis, visual field defects, cortical blindness, visual, ocular motor nerve palsies and pupillary abnormalities. Ho et al, employing virus isolation studies in the cerebrospinal fluid, brain, spinal cord, and peripheral nerves, demonstrated that HIV played an integral role in the neural dysfunction accompanying AIDS. Multiple cranial neuropathy with myelopathy and meningitis can also be due to HIV infection.
CMV infection usually occurs when CD4 count is below 50. In our case CD4 count was relatively higher. Fekrat and associates reported two cases of CMV retinitis with CD4+ T lymphocyte counts of more than 200 cells/mm. In a study of 100 patients with AIDS we had one patient of CMV retinitis with elevated CD4 T cell count. Our report indicates that multiple cranial nerve palsy can be a presenting feature of AIDS. The number of AIDS cases are on the increase in India. Ophthalmologists should be aware of the varied presentation of AIDS and should have tests for HIV done in relevant cases.
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[Figure - 1], [Figure - 2]