James Jose

Cerebral venous thrombosis presenting as multiple lower cranial nerve palsies.

Cerebral venous thrombosis (CVT) is a well-recognized entity, but its clinical presentation is varied and often mimics many neurological disorders, making it a diagnostic challenge. Cerebral venous thrombosis has a wide spectrum of signs and symptoms, which may evolve suddenly or over weeks. It mimics many neurological conditions such as meningitis, encephalopathy, idiopathic intracranial hypertension, and stroke. Cerebral venous thrombosis presenting as multiple lower cranial nerve palsies, are rarely reported. We describe a pregnant lady who presented with sensorineural deafness of the right ear and paralysis of the 9th, 10th, and 12th cranial nerves on the right side. She was diagnosed to have thrombosis of the right transverse sinus and sigmoid sinus with extension to the jugular vein and confluence of sinuses. She improved with anticoagulant treatment.

Inflammatory pseudotumor of the head presenting with hemiparesis and aphasia.

Inflammatory pseudotumor most commonly occurs in the orbit and produces orbital pseudotumor, but extension into brain parenchyma is uncommon. We report a case of inflammatory pseudotumor involving sphenoid sinus, cavernous sinus, superior orbital fissure, orbital muscle, and intracranial extension into left temporal lobe producing right hemiparesis and wernickes aphasia. The patient improved clinically and radiologically with steroid administration. This paper provides an insight into the spectrum of involvement of inflammatory pseudotumor and the importance of early diagnosis of the benign condition.

Posterior cortical atrophy: A rare visual variant of Alzheimer's disease

evaluating entrapment neuropathies, our patient demonstrates the additional value of ultrasonography in eliciting underlying etiology and site of nerve compression in tarsal tunnel syndrome. Nerve conduction studies are limited in localizing the site of tibial nerve compression which can be within the tarsal tunnel or distal to it.[4] Ultrasound provides direct evidence of nerve compression by demonstrating focal nerve enlargement and change in nerve echogenicity. In our patient, ultrasound localized the tibial nerve pathology at the tarsal tunnel due to a synovial cyst attached to flexor hallucis tendon. Our patient serves as an example how conventional nerve conduction testing can be complimented by adding ultrasound evaluation, providing comprehensive information for better treatment planning. A. K. Therimadasamy, R. C. Seet1, Y. H. Kagda2, E. P. Wilder-Smith1 Neurology Diagnostic Laboratory, 1Division of Neurology and 2Department of Orthopaedic Surgery, National University Hospital, Singapore E-mail: mdcwse@nus.edu.sg

Postpartum Woman With Seizures

A 27-year-old woman, postpartum day 9, presented with sudden onset of headache, vomiting, visual blurring, and 3 episodes of generalized tonic-clonic seizures. She had had an uneventful cesarean delivery 9 days previously and was discharged home. Her antenatal period was uneventful, and she did not have gestational hypertension. On examination, she was alert, oriented, and afebrile. Her blood pressure was 150/100 mm Hg, which had increased from her mean antenatal recording of 130/80 mm Hg. Fundus examination result was normal, and there were no focal neurologic deficits. Magnetic resonance imaging (MRI) of the brain showed T2 and T2 fluid-attenuated inversion recovery (FLAIR) hyperintense signals in bilateral parietooccipital lobes (Figures 1 and 2) and bilateral cerebellar hemispheres (Figure 3), without any diffusion restriction. She was treated with intravenous labetalol, nifedipine, and fosphenytoin. Her blood pressure normalized and her symptoms subsided, and she was later discharged.

Acute Necrotizing Vasculitic Neuropathy due to Polyarteritis Nodosa

Necrotizing vasculitic neuropathy in polyarteritis nodosa can rarely present acutely and may mimic acute inflammatory neuropathies. A 53-year-old male presented with an acute neurological illness characterized by paresthesia and weakness of both lower limbs lasting six-days. He also had mild paresthesia of both hands. On examination, there were confluent, purpuric, and ecchymotic patches over the extensor aspects of both lower limbs, which were palpable. Neurological examination revealed grade II/V power with hypotonia and absent reflexes in the lower limbs. All modalities of sensation were decreased below the knee. Sensory impairment was also noted on the fingertips of both hands. Nerve conduction study suggested an asymmetrical sensorimotor axonal neuropathy. Sural nerve biopsy was consistent with necrotizing vasculitis. He was treated with intravenous methylprednisolone followed by oral prednisolone and monthly cyclophosphamide injection for six-months and made a good recovery.

Herpes Simplex Virus Encephalitis in a Healthy Lady

The case describes a middle aged lady with herpes simplex encephalitis. The highlight of the case is the MRI which depicts all the typical features of herpes encephalitis. Herpes virus is the most common cause of encephalitis worldwide. It is very important to recognize this typical MRI picture and initiate Acyclovir therapy at the earliest because, early initiation of therapy is the single factor which affects the prognosis of this serious neurological illness.

Progressive dementia with seizures in an HIV infected lady

A 40-year-old female presented with progressive memory loss over the past 1-month. She had one episode of generalized tonic clonic seizure 2-months back, but did not take any treatment. She had significant involuntary weight loss. On examination she was emaciated and had a BMI of 16.4. Neurological examination showedcognitive decline in the form of loss of recent memory,judgment and abstract thinking, with preserved language and visuospatial orientation. There were no focal neurological deficits. She was tested positive for HIV. Her CD4+ count was 21/µL. Computed tomographyof the brain revealed bilateral hypodensities in the periventricular area (A). MRI of the brain showed bilateral symmetrical periventricular white matter FLAIR hyperintensities in frontal and parietal lobes with diffuse brain atrophy suggestive of HIV encephalopathy (B). She was started on combination antiretroviral therapy with tenofovir, lamivudine and efavirenz and discharged. HIV encephalopathy refers to neurocognitive disorders associated with HIV infection. It usually occurs in late stages of HIV-infection and correlates with advanced immunosuppression. Earliest recognizable findings include impairment of attention and concentration. This progresses to frank dementia, personality changes and motor abnormalities. Neuroimaging typically shows global cerebral atrophy with symmetrical white matter hyperintensities predominantly in the periventricular area. Lesions of progressive multifocal leukoencephalopathy can mimic HIV encephalopathy, but are usually asymmetric, involve the peripheral “U” fibres first and have predilection for parieto-occipital lobes.Treatment of HIV encephalopathy is initiation or intensification of anti-retroviral therapy, and psychomotor improvement is usually seen after therapy.

Long-term response of cerebrospinal fluid pressure in patients with idiopathic intracranial hypertension – A prospective observational study

Background: Idiopathic intracranial hypertension (IIH) is increased intracranial pressure (ICP) with normal cerebrospinal fluid (CSF) contents, in the absence of an intracranial mass, hydrocephalus, or other identifiable causes. The current knowledge of the treatment outcome of IIH is limited, and the data on the natural history of this entity are scant. Objective: The objective of the study is to study the treatment response of IIH by serially measuring the CSF opening pressure and to delineate the factors influencing the same. Materials and Methods: A prospective observational study in a cohort of fifty patients with IIH in whom CSF opening pressure was serially measured at pre-specified intervals. Results: The mean CSF opening pressure at baseline was 302.4 ± 51.69 mm of H2O (range: 220–410). Even though a higher body mass index (BMI) showed a trend toward a higher CSF opening pressure, the association was not significant (P = 0.168). However, the age of the patient had a significant negative correlation with the CSF pressure (P = 0.006). The maximum reduction in CSF pressure occurred in the first 3 months of treatment, and thereafter it plateaued. Remission was attained in 12 (24%) patients. BMI had the strongest association with remission (P = 0.001). Conclusions: In patients with IIH, treatment response is strongly related to BMI. However, patients with normal BMI are also shown to relapse and hence should have continuous, long-term follow-up. The reduction in CSF pressure attained in the first 3 months could reflect the long-term response to treatment.

Idiopathic intracranial hypertension with spontaneous cerebrospinal fluid leak into the orbit

from the posterior end of the optic nerve sheath. The high intracranial pressure might have caused the CSF around the optic nerve to leak and form an encapsulated collection outside the optic nerve. CSF leaks are well described in IIH. Spontaneous CSF rhinorrhea and otorrhea can occur in IIH.[1,2] Such leaks provide a natural mechanism for reducing intracranial pressure, and papilledema is much less common in such cases. In our case, the leak was not manifested exteriorly but internally into the orbit. Optic nerve fenestration is one of the surgical treatments of IIH to reduce the risk of visual loss.[3] We can consider our case as a natural, spontaneous fenestration where CSF escaped A 50-year-old male with chronic inflammatory demyelination polyneuropathy (CIDP) on treatment with deflazacort for over a year presented with insidious onset headache of 3-month duration. The headache was diffuse and was not aggravated by coughing or straining. He did not have any early morning headache, transient visual obscurations, or tinnitus. He was diagnosed to have CIDP 1-year back and had a good response to deflazacort and was advised to continue the treatment. Physical examination revealed swelling of the superolateral aspect of both eyeballs. There was no proptosis, chemosis, or restriction of extraocular movements. Fundus examination showed bilateral papilledema. The magnetic resonance imaging of brain showed linear tortuous T1 hypointense and T2 hyperintense lesion in both orbits [Figure 1]. The lesion was encapsulated, running parallel to the optic nerve in the extraconal space and was connected to the optic nerve sheath near the orbital apex [Figure 2]. There was also a partial empty sella. A lumbar puncture measured an opening pressure of 240 mm of cerebrospinal fluid (CSF). CSF protein was 84 mg/dl, sugar 68 mg/dl, without any cells, and culture was sterile. The patient had immediate relief of headache after removal of 20 ml of CSF. The usual causes of idiopathic intracranial hypertension (IIH) such as obesity, antibiotic use, autoimmune disorders, and thyroid dysfunction were ruled out. A diagnosis of IIH secondary to chronic steroid use was made.

Acute stroke-like presentation of acquired hepatocerebral degeneration.

Neurological manifestations in liver diseases have been well-described. Parkinsonism developing in cirrhotic patients is a unique clinical, neuroradiological, and biological entity. The symptoms are often insidious in onset and occur after liver disease has made its presentation. Acute dysarthria as the presenting manifestation of cirrhosis is rare. Here we report three cases where liver disease made an unusual presentation as acute dysarthria. In all cases the abruptness of the onset prompted the treating physicians to make a diagnosis of stroke. The computed tomography (CT) scans of all these patients did not show any evidence of stroke. This was followed by magnetic resonance imaging (MRI) which showed the characteristic symmetric high-signal intensities in globus pallidus and substantia nigra in T1-weighted images, a reflection of increased tissue concentrations of manganese that helped in making a retrospective diagnosis of liver disease, confirmed later by altered serum albumin to globulin ratios and altered liver echo texture in ultra sonogram.