Laryngeal carcinoma presenting as polymyositis: A paraneoplastic syndrome.

Laryngeal carcinoma is rarely associated with paraneoplastic syndrome. Inflammatory myopathy presenting as paraneoplastic event is commonly associated with carcinomas of ovary, lung, pancreas, stomach, colorectal, and non-Hodgkin's lymphoma. We report a case of elderly male, who presented with proximal muscle weakness and found to be associated with laryngeal carcinoma. Diagnosis of polymyositis (PM) was confirmed based on clinical features, laboratory test, and muscle biopsy. Exclusion of other commonly associated malignancies was done. This patient improved gradually after 6 months of immunosuppressive therapy and management of underlying cancer.

Segmental neuromyotonia.

Unilateral focal neuromyotonia has been rarely reported in fingers or extraocular muscles. We report a case of segmental neuromyotonia in a 20-year-old boy who presented to us with intermittent tightness in right upper limb. Electromyography revealed myokymic and neuromyotonic discharges in proximal as well as distal muscles of the right upper limb. Patient's symptoms responded well to phenytoin therapy. Such an atypical involvement of two contiguous areas of a single limb in neuromyotonia has not been reported previously. Awareness of such an atypical presentation of the disease can be important in timely diagnosis and treatment of a patient.

Neurological manifestations of dengue infection: a review.

Dengue is a common arboviral infection in tropical and sub-tropical areas of the world transmitted by Aedes mosquitoes and caused by infection with one of the 4 serotypes of dengue virus. Neurologic manifestations are increasingly recognised but the exact incidence is unknown. Dengue infection has a wide spectrum of neurological complications such as encephalitis, myositis, myelitis, Guillain-Barré syndrome (GBS) and mononeuropathies. Encephalopathy is the most common reported complication. In endemic regions, dengue infection should be considered as one of the aetiologies of encephalitis. Even for other neurological syndromes like myelitis, myositis, GBS etc., dengue infection should be kept in differential diagnosis and should be ruled out especially so in endemic countries during dengue outbreaks and in cases where the aetiology is uncertain. A high degree of suspicion in endemic areas can help in picking up more cases thereby helping in understanding the true extent of neurological complications in dengue fever. Also knowledge regarding the various neurological complications helps in looking for the warning signs and early diagnosis thereby improving patient outcome.

Neurologic complications in dengue virus infection: a prospective cohort study.

OBJECTIVE: This study aimed to evaluate the incidence and clinical spectrum of neurologic complications, predictors of central and peripheral nervous system involvement, and their outcome in patients with dengue virus infection (DENV). METHODS: To determine the extent of neurologic complications, we used a hospital-based prospective cohort study design, which included laboratory-confirmed cases of dengue and follow-up for 3 months. We also analyzed clinical and laboratory data to assess predictors of neurologic involvement. RESULTS: The study included enrollment of 486 cases. Two were lost to follow-up and excluded. Forty-five patients developed neurologic complications. Of these, 28 patients had CNS and 17 had peripheral nervous system (PNS) involvement, representing an incidence rate for neurologic complications of 9.26%. Significant predictors of CNS involvement were higher mean body temperature (p = 0.012), elevated hematocrit (p = 0.009), low platelet count (p = 0.021), and liver dysfunction (p < 0.001). Predictors of PNS involvement were higher mean body temperature (p = 0.031), rash (p = 0.002), and elevated hematocrit (p < 0.001). The mortality rate was 4.5%. The remainder of the patients recovered. CONCLUSION: An increasingly wide spectrum and higher incidence of neurologic complications of DENV are reported. Clinical and laboratory parameters such as higher mean body temperature, rash, increases in hematocrit, thrombocytopenia, and liver dysfunction are independent predictors of neurologic complications.

Teaching neuroimages: neurocysticercosis with subretinal cyst.

A 30-year-old woman presented with headache and diminution of vision of 2 weeks' duration. Visual acuity was finger counting at 1 meter in the right eye. Fundus examination showed a subretinal cyst (figure 1A). Neurologic examination was normal. Neuroimaging revealed neurocysticercosis (figure 2A). Ultrasound orbit showed subretinal cysticercosis (figure 2B). The patient was started on steroids and laser photocoagulation was recommended.

Acute confusional migraine: a variant not to be missed.

A middle age woman who had frequent migraines was admitted with memory loss following severe unilateral headache, restlessness and confusion. Investigations including haematology, biochemistry, cerebrospinal fluid analysis serology and imaging were normal. As she had frequent migraines with a history of ophthalmoplegic migraine, acute confusional migraine was thought of as a possibility. The patient responded dramatically to intravenous sodium valproate. We report this case to emphasise that acute confusional migraine, a rare variant of migraine, is occasionally encountered in adults and prompt recognition will lead to appropriate management.

Hepatic myelopathy: an unusual neurological complication of chronic liver disease presenting as quadriparesis.

Hepatic myelopathy is a rare neurological complication of chronic liver disease, which is usually seen in adults, presenting as pure motor spastic paraparesis. It is almost always associated with portosystemic shunts and hepatic decompensation. We report a rare case of a young adult man presenting as rapidly progressive spastic quadriparesis because of hepatic myelopathy and associated spontaneous splenorenal shunt and early hepatic decompensation.

Pathological fractures as an initial presentation of Wilson's disease.

Wilson's disease (WD) has varied phenotypic presentations. Here we report the case of a 16-year-old boy who presented with a history of multiple pathological fractures, severe joint deformities, hepatic dysfunction, cognitive decline and limb dystonia. On examination, the patient had pinched out facies, pallor and leukonychia totalis. Bilateral Kayser Fleischer (KF) ring was present. Musculoskeletal examination revealed pectus carinatum, bilateral genu valgus and gun-stock deformity of the left elbow joint. Splenomegaly and moderate ascites were present. Neurological examination revealed mild rigidity and intermittent episodes of dystonic posturing of all four limbs. On this basis a diagnosis of WD with dystonia with cirrhosis of liver with portal hypertension with renal tubular acidosis with renal rickets was thought likely. Investigations confirmed the diagnosis. The patient was started on treatment but he did not improve. He suffered aspiration pneumonia during his hospital stay and succumbed to the illness.

Isolated thalamic tuberculoma presenting as ataxic hemiparesis.

Lacunar syndrome is a neurodeficit secondary to a deep cerebral lesion, usually because of microatheroma of small arteries. Ataxic hemiparesis (AH) is a lacunar syndrome with unilateral pyramidal weakness and ipsilateral ataxia. Thalamic tuberculoma, as a cause of AH, has not been previously described in the literature. We describe an elderly man who presented with left hemiparesis and ipsilateral ataxia. Clinical examination revealed upper motor neuron left facial paresis and left-sided hemiparesis. The patient had incoordination in left upper and lower limbs. Mantoux test was positive and erythrocyte sedimentation rate was elevated. MRI of brain showed a conglomerated hypointense lesion in the right thalamus with a peripheral hyperintensity on T1-weighted imaging and a hyperintense lesion in T2-weighted imaging with significant perilesional oedema, suggesting a tuberculoma. The patient was treated with antitubercular therapy and was symptomatically better at the 9 months follow-up.

Creutzfeldt-Jakob disease presenting with visual symptoms: a case of the 'Heidenhain variant'.

Creutzfeldt-Jakob disease (CJD) belongs to a group of prion diseases that may be caused by the abnormal folding of proteins called prion proteins. The 'Heidenhain variant' is a subclass of patients with CJD, who present with isolated visual symptoms at the onset without any cognitive decline. Here we report such a case of an elderly man presenting with progressive diminution of vision, forgetfulness, abnormal behaviour, myoclonic jerks and akinetic mutism since the last 5 months. On clinical examination, lead pipe rigidity was present in all four limbs, and plantars were bilateral extensors. In view of rapidly progressive dementia associated with myoclonus, a possibility of CJD was entertained. As visual symptoms preceded dementia, hence the Heidenhain variant was strongly suspected. MRI of the brain revealed cortical ribboning, and EEG showed periodic triphasic waveforms with background slowing. The patient succumbed to the illness within 1 month of hospitalisation.

Thalamic syndrome as the heralding manifestation of atlantoaxial dislocation.

In India, Atlantoaxial dislocation (AAD) is the commonest skeletal craniovertebral junction (CVJ) anomaly, followed by occipitalisation of atlas and basilar invagination. The usual presentation is progressive neurological deficit (76-95% cases) involving the high cervical cord, lower brainstem and cranial nerves. The association between vertebro-basilar insufficiency and skeletal CVJ anomalies is well recognised and angiographic abnormalities of the vertebrobasilar arteries and their branches have been reported; however, initial presentation of CVJ anomaly as thalamic syndrome due to posterior circulation stroke is extremely rare. Here, we report one such rare case of thalamic syndrome as the initial presentation of CVJ anomaly with AAD.

Spastic foot-drop as an isolated manifestation of neurocysticercosis.

Foot-drop is a rare but important manifestation of intracranial pathologies ranging from space-occupying lesions to cerebrovascular accidents. Being most commonly associated with peripheral nerve lesions or radicular compressions, it remains an underappreciated feature of central-structural abnormalities. We describe an interesting case of a 14-year-old boy who had presented with acute onset right-sided foot-drop due to a left-sided parasagittal neurocysticercus lesion, without seizures and discuss the location of the lesion in the precentral area in reference to Penfield's motor homunculus.

Hypothyroid myopathy mimicking postpolio syndrome.

Hypothyroidism can have diverse neurological manifestations. Myopathy may rarely be the sole manifestation of autoimmune thyroiditis. We hereby report an atypical manifestation of severe hypothyroidism in a middle-aged woman with childhood onset of paralytic polio involving her right leg presenting with a recent onset of increased weakness in the right leg mimicking postpolio syndrome.

Purple glove syndrome: a dreadful complication of intravenous phenytoin administration.

Purple glove syndrome is an uncommon but dreaded complication of intravenous phenytoin administration characterised by pain, oedema and purple-blue discolouration of the limb distal to the site of injection. We describe a 37-year-old gentleman having the characteristic purple glove appearance after phenytoin loading, and discuss the salient features of this syndrome highlighting the pathophysiological and preventive aspects.

Subarachnoid haemorrhage as the initial manifestation of cortical venous thrombosis.

Aneurysmal rupture is the commonest cause of non-traumatic subarachnoid haemorrhage (SAH). SAH can rarely be the manifestation of cortical venous thrombosis (CVT). CVT is potentially lethal but treatable disorder with positive outcome if timely treatment is instituted. The site of bleeding is mainly on convexities and sulcus with sparing of basal cisterns in SAH related to CVT. Anticoagulation is the mainstay of treatment, in spite of SAH complicated by CVT. In this submission, the author highlighted a case of SAH presented as initial manifestation of CVT in an elderly woman. Early therapy with anticoagulation led to complete clinical and radiological recovery in a short duration of 2 weeks. Thus, diagnosis of CVT should be kept in mind in unusual presentation of SAH.