Geeta Anjum Khwaja

Acute disseminated encephalomyelitis associated with dengue infection: a case report with literature review.

Dengue is the commonest arboviral illness caused by four antigenically distinct dengue virus serotypes (DEN-1 through DEN-4). The clinical spectrum of the disease ranges from asymptomatic or mild infection to catastrophic dengue shock syndrome (DSS). In last few years, neurological manifestations of dengue infection have been increasingly observed and reported mainly with serotypes DEN-2 and DEN-3. The pathogenesis of neurological manifestations includes: neurotrophic effect of the dengue virus, related to the systemic effects of dengue infection, and immune mediated. Encephalopathy and encephalitis are the most frequently reported neurological manifestations followed by meningitis, myositis, hypokalemic periodic paralysis, stroke, Guillain-Barré syndrome and transverse myelitis. Acute disseminated encephalomyelitis (ADEM) associated with dengue infection is rarely reported. We herein report a case of ADEM associated with classic dengue fever. Favourable clinical outcome occurred after a five-day course of intravenous methylprednisolone therapy.

Behavioural and psychological symptoms in poststroke vascular cognitive impairment.

Background. Behavioural and psychological symptoms of dementia (BPSD) cause significant patient and caregiver morbidity in vascular cognitive impairment (VCI). Objectives. To study and compare the occurrence and severity of BPSD between multi-infarct dementia (MID), subcortical ischaemic vascular disease (SIVD), and strategic infarct subtypes of poststroke VCI and to evaluate the relationship of these symptoms with the severity of cognitive impairment. Methods. Sixty patients with poststroke VCI were classified into MID, SIVD, and strategic infarct subtypes. BPSD were studied by the neuropsychiatric inventory (NPI). The severity of cognitive impairment was evaluated by the clinical dementia rating scale (CDR). Results. 95% of cases had at least one neuropsychiatric symptom, with depression being the commonest, irrespective of subtype or severity of VCI. Strategic infarct patients had the lowest frequency of all symptoms. SIVD showed a higher frequency and severity of apathy and higher total NPI scores, compared to MID. Apathy and appetite disturbances occurred more commonly with increasing CDR scores. The total NPI score correlated positively with the CDR score. Conclusion. Depression was the commonest neuropsychiatric symptom in VCI. The neuropsychiatric profiles of MID and SIVD were similar. The frequency and severity of apathy and the net burden of BPSD increased with increasing cognitive impairment.

Epilepsy and religion

This study has focused on the interplay between epilepsy and religion. A total of 100 patients in the age range of 15-84 years were included in the study. The duration of epilepsy in these patients ranged from 1-35 years. The majority (66%) had generalized seizures and good to complete seizure control (77%). Regarding social/religious beliefs, 6% of the patients attributed their epilepsy to the curse of God and 14% saw their affliction as a form of punishment for bad deeds committed in the current or past life. Epilepsy was regarded as contagious by 13%. After the onset of epilepsy, 7% of the subjects became skeptics and less religious, while 29% became more religious. Only 2% reported mystic experiences. There was, however, no significant impact of the duration of epilepsy or seizure type on the pattern of religiosity. In 44 cases with symptomatic epilepsy, no definite correlation was observed between the lesion site and laterality and the religious temperament. Delay in seeking treatment and poor compliance due to false religious beliefs, ignorance, and superstition was observed in 33%. However, all religious beliefs were not maladaptive and overall, 80% cases felt that religion had helped them in coping with epilepsy.

The profile of behavioral and psychological symptoms in vascular cognitive impairment with and without dementia

Objective: The objective of this study was to compare the occurrence and severity of behavioral and psychological symptoms of dementia (BPSD) between vascular dementia (VaD) and vascular cognitive impairment-no dementia (VCI-ND). Materials and Methods: Consecutive patients presenting with cognitive impairment at least 3 months after an ischemic stroke and with a Hachinski Ischemic Score ≥4 were included. VaD was diagnosed as per National Institute of Neurological Disorders and Stroke – Association Internationale pour la Recherche et l’Enseignement en Neurosciences criteria for probable VaD and VCI-ND on the lines of the Canadian study of health and aging. The severity of cognitive impairment and the behavioral/psychological symptoms were studied by means of the clinical dementia rating scale and the neuropsychiatric inventory (NPI) respectively. Results: All patients with VaD and 89% of those with VCI-ND had at least one BPSD. The mean no. of symptoms per patient and the total NPI scores were higher in VaD than in VCI-ND. Apathy and night-time behavior disturbances were significantly more common and severe in VaD. Conclusions: BPSD are very common both in VCI-ND and in VaD. The profile of BPSD is similar in both groups, albeit more severe in VaD. The net burden of BPSD is higher in VaD as compared to VCI-ND.

Clinical profile of psychogenic non-epileptic seizures in adults: A study of 63 cases

Aims: To evaluate clinical profile and short-term outcome of psychogenic non-epileptic seizures (PNES) in Indian adult population. Setting and Design: A prospective observational study, conducted at tertiary teaching institute at New Delhi. Materials and Methods: Sixty-three patients with confirmed PNES were enrolled. The diagnosis was based on witnessing the event during video-electroencephalography (Video-EEG) monitoring. A detailed clinical evaluation was done including evaluation for coexistent anxiety or depressive disorders. Patients were divided into two groups on the basis of excessive or paucity of movements during PNES attacks. Patients were followed-up to 12 months for their PNES frequency. Statistical Analysis: Means and standard deviations were calculated for continuous variables. Chi-square and Students t-test were used to compare categorical and continuous variables respectively. Results: The mean age at onset of PNES was 25.44 years; with F:M ratio of 9.5:1. Coexistent epilepsy was present in 13 (20.63%) cases. Twenty-two patients (44%) with only PNES (n = 50) had received antiepileptic drugs. Out of 63 patients of PNES 24 (38.1%) had predominant motor phenomenon, whereas 39 (61.9%) had limp attacks. The common features observed were pre-ictal headache, ictal eye closure, jaw clenching, resistant behavior, ictal weeping, ictal vocalization, and unresponsiveness during episodes. Comorbid anxiety and depressive disorders was seen in 62.3% and 90.16% patients, respectively. Short-term (6-12 months) outcome of 45 patients was good (seizure freedom in 46.66% and >50% improvement in 24.44% cases). Conclusion: PNES is common, but frequently misdiagnosed and treated as epileptic seizures. A high index of suspicion is required for an early diagnosis. Proper disclosure of diagnosis and management of the psychiatric comorbidities can improve their outcome. Limitation: Limited sample size and change in seizures frequency as the only parameter for the assessment of the outcome are the two major limitations of our study.

A case of crossed aphasia with apraxia of speech

Apraxia of speech (AOS) is a rare, but well-defined motor speech disorder. It is characterized by irregular articulatory errors, attempts of self-correction and persistent prosodic abnormalities. Similar to aphasia, AOS is also localized to the dominant cerebral hemisphere. We report a case of Crossed Aphasia with AOS in a 48-year-old right-handed man due to an ischemic infarct in right cerebral hemisphere.

Paroxysmal dystonic choreoathetosis with symptomatic seizures secondary to hypoglycemia caused by insulinoma

Neurological syndromes are not an uncommon presentation with insulinomas. Recurrent hypoglycemia associated with it can present with a variety of neurological symptoms that may include disturbances of consciousness, seizures, stroke-like presentation, movement disorder, dementia and chronic neuropathy. The myriad of presentations, resemblance with other neurological conditions and episodic nature often lead to misdiagnosis and a delay in definitive treatment. Rare cases of insulinoma presenting as combination of abnormal movements have been described. We report a patient who presented with both hypoglycemia induced symptomatic seizures and paroxysmal non-kinesiogenic dystonic choreoathetosis. Insulinoma is a potentially treatable disorder and early definitive intervention can prevent long term neurological disability in patients.

Gluten Sensitivity - A Potentially Reversible Cause of Progressive Cerebellar Ataxia and Myoclonus - A Case Report.

Gluten sensitivity is an umbrella term used for diverse clinical manifestations occurring as a result of abnormal immunological reactivity to dietary gluten in genetically susceptible individuals. Celiac disease is the most well-known but not the only manifestation of gluten sensitivity. Myoclonus with Ataxia is a rare manifestation of gluten sensitivity and should be considered in the differential diagnosis of all patients with idiopathic sporadic ataxia. The presence of gluten-related immune markers in normal population however complicates the reliable diagnosis of gluten related neurological disorders and clinical improvement on gluten free diet can serve as a diagnostic tool for this disease. We report a case of sporadic progressive cerebellar ataxia with myoclonus with positive antigliadin antibodies, which improved with a trial of gluten free diet. This case highlights an important diagnostic and therapeutic principle in management of late onset idiopathic ataxia.

Isolated lingual involvement in Wilson's disease

Lingual involvement can occur in a variety of neurological disorders including pyramidal, extrapyramidal and lower motor neuron disorders. It can be seen in the form of tremor, bradykinesia, dystonia, atrophy and weakness of tongue movements and can clinically present as difficulty in swallowing and dysarthria which can be a source of great discomfort to the patient. We describe a patient who presented with isolated lingual involvement and was diagnosed to have Wilsonss disease. This case emphasizes the clinical variability in presentation of Wilsons disease and importance of early clinical diagnosis.

Pregnancy related symptomatic vertebral hemangioma

Vertebral hemangiomas are benign vascular tumors of the spine that remain asymptomatic in most cases and incidentally encountered on imaging. Rarely, altered hemodynamic and hormonal changes during pregnancy may expand these benign lesions resulting in severe cord compression. The management of symptomatic vertebral hemangioma during pregnancy is controversial as modalities like radiotherapy and embolization are not suitable and surgery during pregnancy has a risk of preterm labor. Few cases of pregnancy related symptomatic vertebral hemangioma with marked epidural component have been reported in the literature. We report a case of 23-year-old primigravida who developed rapidly progressive paraparesis at 28 weeks of gestation and spine magnetic resonance imaging (MRI) revealed upper thoracic vertebral hemangioma with extensive extra-osseous extension and spinal cord compression. Laminectomy and surgical decompression of the cord was performed at 32 weeks of the pregnancy. There was significant improvement in muscle power after a week of surgery. Six weeks postoperatively she delivered a full term normal baby with subsequent improvement of neurologic deficit. Repeat MRI of dorsal spine performed at 3 months postoperatively showed reduced posterior and anterior epidural components of vertebral hemangioma.