Boby Varkey Maramattom

Acute flaccid paralysis due to West nile virus infection in adults: A paradigm shift entity

Three cases of acute flaccid paralysis (AFP) with preceding fever are described. One patient had a quadriparesis with a florid meningoencephalitic picture and the other two had asymmetric flaccid paralysis with fasciculations at the onset of illness. Magnetic resonance imaging in two cases showed prominent hyperintensitities in the spinal cord and brainstem with prominent involvement of the grey horn (polio-myelitis). Cerebrospinal fluid (CSF) polymerase chain reaction was positive for West Nile virus (WNV) in the index patient. All three cases had a positive WNV immunoglobulin M antibody in serum/CSF and significantly high titer of WNV neutralizing antibody in serum, clearly distinguishing the infection from other Flaviviridae such as Japanese encephalitis. WNV has been recognized in India for many decades; however, AFP has not been adequately described. WNV is a flavivirus that is spread by Culex mosquitoes while they take blood meals from humans and lineage 1 is capable of causing a devastating neuro-invasive disease with fatal consequences or severe morbidity. We describe the first three laboratory confirmed cases of WNV induced AFP from Kerala and briefly enumerate the salient features of this emerging threat.

N-methyl D-aspartate receptor encephalitis: A new addition to the spectrum of autoimmune encephalitis

A large proportion of “encephalitis” is caused by unknown agents. Of late, a new category of disorders, “autoimmune encephalitis,” has been described, which present with features similar to viral encephalitides. A well-delineated and common entity among this group is the recently described anti-NMDAR encephalitis (NMDARE). Although this entity was initially described in young women harboring ovarian teratomas, it is now characterised as well in children and men. Approximately 60% of the patients have an underlying tumor, usually an ovarian teratoma. In 40% of the patients, no cause can be found (idiopathic NMDARE). NMDARE typically presents with psychiatric features followed by altered level of consciousness, severe dysautonomia, hyperkinetic movement disorders, seizures and central hypoventilation. Orofacial dyskinesias resulting in lip and tongue mutilation are quite common. Seizures, are common and may be difficult to treat. The disease can be confirmed by serum and cerebrospinal fluid anti-NMDAR antibodies. Titers of these antibodies can also guide response to treatment. Tumor removal is necessary if identified, followed by immunological treatment. Intravenous methylprednisolone and immunoglobulins aim to suppress/modulate immune response while plasma exchange attempts to remove antibodies and other inflammatory cytokines. Rituximab and cyclophosphamide aim to suppress antibody production. Recovery is slow and often with neurological deficits if treatment is delayed. With many distinctive clinical features, a specific antibody that aids diagnosis, and early effective treatment with commonly available drugs leading to good outcomes, NMDARE is a diagnosis that should be considered early in any case of “unexplained encephalitis.”

Cerebral hyperperfusion syndrome after intracranial stenting of the middle cerebral artery

Cerebral hyperperfusion syndrome (CHS) is a rare complication following cerebral revascularization. It presents with ipsilateral headache, seizures, and intracerebral hemorrhage. It has mostly been described following extracranial carotid endarterectomy and stenting and it is very unusual after intracranial stenting. A 71-year-old man with a stuttering stroke was taken up for a cerebral angiogram (digital subtraction angiography), which showed a dissection of the distal left middle cerebral artery. This was recanalized with a solitaire AB stent. After 12 h, the patient developed a right hemiplegia and aphasia. Computed tomography brain showed two discrete intracerebral hematomas in the left hemisphere. This is the first reported case of CHS following intracranial stenting from India.

Facio-brachio-crural dystonic episodes and drop attacks due to leucine rich glioma inactivated 1 encephalitis in two elderly Indian women.

Two women in their 60s are presented to us with sudden falls of acute onset. Prolonged observation revealed a gradually evolving syndrome of paroxysmal right sided faciobrachial dystonic (FBD) posturing lasting seconds. Both patients went on to develop hyponatremia, following which the episodes worsened and appeared on both sides. In both cases, prolonged electroencephalography monitoring and magnetic resonance imaging brain were normal and the response to conventional anticonvulsants was poor. One patient improved spontaneously over 6 months. The 2nd patient developed an amnestic syndrome and was started on intravenous methylprednisolone with which her movement disorder abated. Her amnestic syndrome improved and she was discharged on oral steroids. Both patients tested positive for leucine-rich glioma inactivated 1 (LGi1) antibodies. We present the first case reports of FBD episodes and drop attacks owing to LGi1 encephalitis from India and review the relevant literature pertinent to the subject.

Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome

Severe muscle rigidity and spasms are uncommon causes of Intensive Care Unit (ICU) admissions. Stiff-man syndrome (SMS) is a rare disorder characterized by continuous muscle spasms, axial muscle rigidity, “tin soldier gait,” and continuous motor unit activity on electromyography. There are three clinical variants of SMS; stiff-limb syndrome, classical SMS, and paraneoplastic encephalomyelitis with rigidity and myoclonus. Three types of antibodies have been associated with SMS; however, anti-glutamic acid decarboxylase (GAD) antibodies are the most frequent and are seen in the idiopathic type of SMS. The spasms of SMS can be very disabling and severe enough to cause muscle ruptures and skeletal fractures. We present a case of anti-GAD positive SMS with “status spasticus” causing bilateral psoas myoedema and rhabdomyolysis due to repeated axial muscle jerking in a 64-year-old man and discuss the differential diagnosis of a “jerking patient in the ICU.”

Paraneoplastic CRMP-5 basal ganglionitis and limbic encephalitis in an elderly Indian lady

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Clinical features and outcome of acute disseminated encephalomyelitis (ADEM): An outlook from South India

Introduction: Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating encephalomyelitis that may follow infections, vaccinations or occur spontaneously. Most of the large series of this disorder were published in the pre-MRI era. Subsequently there has been a paucity of data regarding this entity. Aims: We sought to describe our experience with ADEM across 2 hospitals from Kerala, Sree chitra tirunal institute of medical sciences, thiruvanthapuram and the Indo-american Brain and spine center, Vaikom. We wanted to look at the clinico-radiological parameters of this patient population as well as the functional outcome following ADEM. Materials and Methods: A total of 45 patients seen in these two centers over a period of 9 years from 1995 to 2003 were analyzed in a retrospective-prospective design. MRI, CT scans, laboratory investigations, nerve conduction parameters and modified rankin outcome scores at last follow up were also noted. Results: The clinico-radiological profile of our patients was comparable to that of patients described in the literature. Relapse was uncommon although transient reappearance of prior symptoms during subsequent illness was common. Possible multiple sclerosis could be diagnosed only in one patient during follow up. Mortality was low ( Conclusions: ADEM deserves to be distinguished from MS in our population as there seems to be a low likelihood of recurrence or relapse. Although mortality rates have improved greatly, survivors are left with a plethora of disabilities and are functionally impaired. Future studies should focus on specific disabilities and treatment options to further improve outcomes in ADEM

Postoperative posterior ischemic optic neuropathy (PION) following right pterional meningioma surgery.

Postoperative visual loss (POVL) is an unpredictable complication of nonocular surgeries. Posterior ischemic optic neuropathy (PION) is particularly feared in spinal surgeries in the prone position. We report a rare case of PION occurring after surgery for a pterional meningioma and discuss the various factors implicated in POVL.

HHV-6 associated fulminant encephalo-myocarditis in an immunocompetent individual.

was positive for Human Herpes Virus 6 [HHV-6] on day 5 and negative for herpes simplex virus (HSV), enteroviruses and other viruses by PCR panel [Xcyton AES, Bangalore]. ECG showed sinus tachycardia, extensive ST-T changes, cardiac biomarkers were elevated [CK-MB 85 U/l, Troponin I, 0.4 U], and echocardiogram showed global hypokinesia suggestive of myocarditis [Video 1]. Increasing dyspnea necessitated mechanical ventilation and he expired on day 7. Primary infection with HHV-6 occurs in >95% of individuals by the age of 2 years. HHV-6-associated encephalitis, limbic encephalitis, or myocarditis are well described in both immunocompromised patients and immuno-competent patients.[1-3] Imaging features suggestive of limbic encephalitis should prompt testing for HHV-6 in addition to HSV or paraneoplastic encephalitis.[4] An encephalo-myocarditis syndrome may also be present.

Montelukast-associated Churg-Strauss syndrome with mononeuritis multiplex.

Montelukast and other leukotriene-receptor antagonists (LTA) use are associated with a 4.5-fold higher risk of CSS.[2] Montelukast is a selective competitive antagonist of cysteinyl leukotriene receptors and increases levels of the leukotriene LTB4 leading to vasculitis by virtue of its potent chemoattractant property for neutrophils and eosinophils. CSS can be diagnosed if at least four out of six American College of Rheumatology (ACR) 1990 criteria are fulfilled; asthma, eosinophilia >10%, mononeuropathy (including multiplex) or polyneuropathy, nonfixed pulmonary infiltrates on chest X-ray, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils.[3] More than four of these criteria had a sensitivity of 85% and a specificity of 99.7% for CSS. Mononeuritis multiplex is one of the most common peripheral nervous system manifestations of CSS and early treatment with steroids can stop disease progression.[4] Our patient fulfilled four of the ACR criteria and improved after drug withdrawal and steroid administration. It is essential to recognize secondary CSS as discontinuation of the offending agent is also imperative. Sir, A 59-year-old man presented to us with numbness of legs of 3 months duration. He had a history of diabetes mellitus since 1 year. He had developed spontaneously resolving hyperpigmentation over forehead, back, hands, and legs and erythematous rashes over the trunk 3 months earlier followed by gradual onset of numbness and weakness in both legs and difficulty in buttoning. Over the last 1 week it had suddenly worsened and became chair-bound. He had a history of late onset asthma (for the past 2 years), which was controlled with inhaled corticosteroids, beta agonists, and a combination of montelukast and cetirizine; and late onset hypothyroidism on treatment with levothyroxine 100 μg. On examination, he had pedal edema and scattered erythematous rashes over back, buttock, right leg, and right forearm without nerve thickening. He had bilateral claw hand and foot drop with grade 0 weakness in both distal legs. There was sensory impairment in right fifth finger, left finger tips, and both lower limbs (LL) upto knees. Blood reports showed the following; hemoglobin (Hb) 11.7 g%, total count (TC) 11,700, eosinophils 14% (absolute eosinophil count 1,500), platelets 300,000, erythrocyte sedimentation rate (ESR) 77 mm/h, C-reactive protein 29mg/L, human immunodeficiency virus (HIV)/hepatitis B surface antigen (HBsAg)/hepatitis C virus (HCV) were negative, perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA)/ cytoplasmic ANCA (C-ANCA) were negative, anti-double stranded deoxyribonucleic acid (dsDNA) was 29.7 IU/ml, rheumatoid arthritis (RA) factor 345 IU/ml, liver function test (LFT) showed albumin-globulin reversal, and creatinine was 0.7 mg%. Urine microscopy showed granular casts with 20-25 red blood cells (RBCs)/high power field (HPF) without proteinuria and HbA1c was 6.4%. Chest X-ray and paranasal examination were normal. Nerve conduction studies showed absent sensory nerve action potentials (SNAPs) in the sural nerves and left ulnar nerve and absent compound muscle action potentials (CMAPs) in both peroneal and right ulnar nerves. The study was suggestive of mononeuritis multiplex.