Vivek Junewar

Bilateral cavernous sinus thrombosis and facial palsy as complications of dental abscess.

Septic cavernous sinus thrombosis (CST) related to dental infection is a rare clinical event. The septic CST is a disease of high morbidity and mortality. The prompt diagnosis and timely treatment of septic CST is cornerstone of successful outcome. The dental infection should be given due attention, as to prevent CST. In this case report of immunocompetent female, we highlighted the role of dental abscess in producing bilateral CST and facial palsy. The close collaboration between dentist and neurologist and early institution of antibiotics led to complete recovery at follow-up after 3 months. The dental infection should never be neglected as it is the interface of serious intracranial complication like CST.

An atypical case of neuroleptic malignant syndrome precipitated by valproate

Neuroleptic malignant syndrome (NMS) can be caused by various drugs. We report a case of a 60-year-old woman who presented with high-grade fever, muscular rigidity, tachycardia, tachypnoea and altered sensorium along with seizures. She had been taking olanzapine for the past 2 years for psychosis. For the last month valproate was added to her treatment. Her blood investigations revealed hyponatraemia and raised serum ammonia and creatinine phosphokinase (CPK) levels. In view of hyperthermia, muscular rigidity, autonomic disturbances, altered mental status and raised CPK, a diagnosis of NMS was made. Valproate could have probably precipitated NMS; although the patient was taking antipsychotics for a long time, it was only with the addition of valproate that she developed these symptoms. Raised serum ammonia levels also indicated the presence of valproate toxicity. Seizures were probably due to electrolyte disturbances. Offending drugs were withdrawn. The patient improved with treatment by dopamine agonist and other supportive treatments.

Pathological fractures as an initial presentation of Wilson's disease

Wilsons 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.

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.

Spinal cord tuberculosis: a paradoxical response to antituberculous therapy

A 22-year-old man, with a known case of definitive tuberculous meningitis (TBM) on antitubercular therapy (ATT) for 5 months, presented with subacute onset sensorimotor paraparesis with urinary urgency since 1 month. He had also received dexamethasone therapy 0.4 mg/kg/24 h for 1 month followed by reducing course over next 2 weeks as per British Infection Society guidelines. MRI of the spine revealed a ring enhancing intramedullary lesion at D4 level suggestive of …

Paroxysmal kinesigenic dyskinesia: a frequently misdiagnosed movement disorder

A 13-year-old boy previously diagnosed as having psychogenic movement disorder presented with a 2-year history of episodic dystonic movements involving all limbs and face. The episodes were precipitated by sudden movements, lasted for less than 30 s, occurring 5–6 times/day and were preceded by tingling sensations (video 1). There was no history of any major medical or surgical illness. Family …

Isolated vision loss and disappearing lesions as the manifestation of subacute sclerosing panencephalitis

Sir, Subacute sclerosing panencephalitis (SSPE) is a devastating disease of central nervous system (CNS) caused by persistent mutant measles virus infection. Although it is a rare disease in developed nations, it is of great concern in developing countries. Patients present with progressive cognitive decline and myoclonus.[1] Visual manifestations are present in 10-50% of patients but they usually occur concurrently with above neurological findings. Rarely visual symptoms may precede neurological manifestations.[2] Here we report a case of SSPE that had presentation in the form of acute vision loss and remained undiagnosed for 2 years until he developed typical manifestations of SSPE.

Unusual association of eclamptic encephalopathy and Japanese encephalitis.

Eclampsia is one of the hypertensive disorders of pregnancy causing considerable maternal morbidity and mortality. It is more common in developing nations, where access to adequate obstetric care is limited to a small population. Eclampsia is clinically characterised by encephalopathy, seizures, headache and cortical blindness and occurs in the setting of pre-eclampsia that comprises hypertension, proteinuria and peripheral oedema. Japanese encephalitis has emerged as a serious neurological disorder in South East Asian countries. In this case report, we highlight the unusual association of eclamptic encephalopathy and Japanese encephalitis in a patient belonging to northern India. This combination of illnesses caused diagnostic difficulty and delayed hospitalisation. A high index of suspicion is required in the endemic zone of Japanese encephalitis if the expected results are lacking in patients with eclampsia.

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.

A rare case of basilar impression

A teenage girl presented with progressively worsening neck and occipital pain since 8 months ago that was associated with restriction of neck movements, low to moderate grade fever, anorexia and weight loss followed by gradually progressive quadriparesis since 3 months ago. Neurological examination revealed spastic quadriparesis without cranial nerve palsy. MRI of the cervical spine revealed prevertebral and paravertebral abscess from clivus to C2/C3 level, which extended into the anterior epidural space at C1–C3 level, causing atlantoaxial dislocation. There was evidence of basilar invagination. The dislocated dens caused compression over the cervicomedullary junction. On this basis, a diagnosis of craniovertebral junction Potts disease was made. The patient was given neck immobilisation with cervical collar, and antitubercular treatment was started. She showed significant improvement in her neurological deficit during follow-up.