Clinical and Radiological Profile of 122 Cases of Idiopathic Intracranial Hypertension in a Tertiary Care Centre of India: An Observational Study.

Background: Idiopathic intracranial hypertension (IIH) is well-described entity in literature. However, large studies on clinical and radiological profile are still very few from the Indian subcontinent. Aims: To analyze the clinical and radiological profile of IIH and correlation of various clinical and radiological parameters with papilledema and CSF opening pressure. Setting and Design: Hospital-based observational, descriptive study. Materials and Methods: This study analyzed 122 patients admitted in the department of Neurology (from January 2014 to December 2018) for detailed history, clinical examination, and neuroimaging. CSF manometry and routine CSF analysis were done. All participants met the modified Dandy criteria. Patients with secondary causes of raised ICP and primary ocular pathology were excluded. Statistical Analysis Used: Descriptive statistics and Chi-square test. Results: Among 122 cases 86 (70.49%) were females. The mean age was 33 years. 62 (50.82%) cases were obese. Headache was the most common symptom (114/122; 93.44%). Bilateral papilledema was noted in 114 (93.44%) cases. Higher grades of papilledema had a correlation with higher CSF opening pressure. Empty sella was the most common radiological finding (100/122; 81.97%). All radiological findings except empty sella had a correlation with higher CSF opening pressure. Conclusion: IIH typically affects obese females of childbearing age group. However, it is not uncommon in the nonobese and male sex. Obesity may not be a dominant risk factor for the development of IIH in the Indian subcontinent. Cases with normal BMI and male sex with clinical features of raised intracranial pressure and normal neuroimaging should also be evaluated for IIH. Cases with refractory headache and empty sella on MRI should be evaluated for IIH.

Neuroborreliosis in India - A Diagnostic Challenge and a Great Mimicker: A Case Series.

OBJECTIVES: Neuroborreliosis is generally known to be a disease confined to the Western part of the globe. It is not commonly encountered in this part of the world. Interestingly, we recently came across a series of cases of Lyme's disease with a plethora of neurological presentations. Most of the cases were a diagnostic dilemma, with poor response to immunotherapy and on subsequent evaluation all were found to have positive Borrelia antibodies. MATERIALS AND METHODS: Eight cases were selected from the tertiary care hospital in North western India. Patients were suspected to have Neuroborreliosis whose neurological presentations were atypical for other classical neurological disorders, who had a progressive or relapsing clinical course and had responded poorly to the initial treatment given for the previous neurological diagnosis. Skin lesions were present in some cases. The patients underwent a detailed clinical assessment which comprised of an elaborate history including history of travel, any insect bite or skin rashes along with a complete systemic and neurological examination. All the required blood investigations, Magnetic Resonance Imaging (MRI) Brain, Computer Tomography Angiography (CT), Nerve conduction study (NCS) and Electromyographic (EMG) studies and Cerebrospinal fluid (CSF) studies were done as indicated in each case. Borrelia antibody titre was done in all the patients using immunoblot technique. RESULTS: Among the 8 patients, 6 were male and 2 were females. The age group was between 25-70 years. The clinical presentation was acute, subacute or chronic. One patient gave a clear history of tick bite. Two patients had skin lesions and one had the pathognomic "eschar". All the suspected 8 patients had either IgG or IgM or both IgG and IgM Borrelia antibodies positive. Almost all the patients had previously received either steroids or intravenous immunoglobulins, but had not adequately responded to immunotherapy. These patients were given a trial of injectable Ceftriaxone and oral Doxycycline. Most of them either showed partial or complete clinical improvement. CONCLUSION: Lyme's disease, a common disease of the west does exist in the Indian subcontinent as well. Because of increasing global travel and migration and change in vector habitat the disease seems to have percolated in the non endemic areas too. Proper history of travel or exposure to tick bite is important. We want to emphasize, Neuroborreliosis, a great mimicker may have diverse and varied neurological presentations and has a potential for reversibility with appropriate treatment even after a significant delay in diagnosis.

Prevalence, Demographic Profile, and Psychological Aspects of Epilepsy in North-Western India: A Community-Based Observational Study.

AIMS: This study was undertaken to determine the prevalence of active epilepsy, assess the sociodemographic profile, and psychological aspects of epilepsy in the Jaipur district of Rajasthan, India. METHODS: We conducted a community-based, cross-sectional observational study covering both rural (n = 165,660) and urban (n = 179,142) populations of Jaipur district using a house-to-house survey. An adapted, pre-designed World Health Organization screening questionnaire was used to identify the cases. Those confirmed by neurologists as true seizures were included in the study. Cases were classified as per the International League against Epilepsy recommendation. Global Mental Health Assessment Tool electronic questionnaire was used to analyze psychological aspects of cases. The caregivers of the patients participated in the knowledge, attitude, and practice (KAP) survey. RESULTS: A total of 380 patients (258 men, 122 women) were identified with active epilepsy. The estimated prevalence of active epilepsy was 1.1/1,000 population and 71% of cases belonged to low socioeconomic classes. Primary treatment gap was documented in 18.8% of cases in our study, 38% of cases were non-compliant to treatment with poorer compliance in those on pol-therapy, 76% had some psychiatric disorder, anxiety and depression being the commonest, and positive family history of epilepsy was found in 4.7%. KAP survey revealed that only 15% of the respondents believed that epilepsy is non-curable, 74% denied its infectious nature, 26% believed that epilepsy occurs due to past sins, and 81% said that they would not marry persons with epilepsy. CONCLUSION: A relatively low prevalence (1.1/1,000) of active epilepsy and a smaller primary treatment gap (18.8%) was found in our study population. Almost three-fourth of cases had an associated psychological problem, Though caregivers were aware of the nature of disease, majority would not prefer to marry a person suffering from epilepsy.

Transient elevation of cerebrospinal fluid protein in a patient of mild encephalitis with reversible lesion in the splenium: a case report.

Mild encephalitis with reversible lesion in the splenium (MERS) is a clinicoradiological syndrome presenting as a solitary lesion in the central portion of the splenium of the corpus callosum (SCC) with a radiological finding of restricted diffusion and low apparent diffusion coefficient (ADC) values. Complete resolution of the lesion on follow-up imaging and full clinical recovery are the hallmarks of this syndrome, even with only supportive therapy. MERS is usually associated with normal Cerebrospinal fluid (CSF) findings and an excellent prognosis, even without corticosteroid therapy. Magnetic resonance imaging (MRI) is the ideal modality for initial diagnosis and follow-up. Not many cases of this uncommon clinicoradiological syndrome with transient elevation of CSF proteins have been reported. In the subsequent sections, we present a case report of this unusual clinicoradiological entity with raised CSF protein. We also elaborate on possible differential diagnoses and the syndrome's proposed pathophysiology.

Scalp fibroma: a rare cutaneous manifestation of tuberous sclerosis.

We report a case of a 23-year-old woman with a history of generalised tonic-clonic seizures, reddish brown maculopapular swelling over the face and an enlarging swelling over the scalp. Physical examinations revealed angiofibroma of the face and other typical cutaneous lesions of tuberous sclerosis, for example, shagreen patch and periungual fibroma. Scalp swelling was labelled as fibroma by dermatologists, which was further supported by the histopathological findings. Fibroma of the face is one of the commonest lesions, however, fibroma of the scalp is a rarely described entity.

Combination of Steven-Johnson syndrome and neuroleptic malignant syndrome following carbamazepine therapy: a rare occurrence.

Stevens-Johnson syndrome (SJS) is a severe, episodic, acute mucocutaneous reaction that is most often elicited by drugs and occasionally by infections. The drugs commonly implicated as the cause of SJS are anticonvulsants, sulfonamides, non-steroidal anti-inflammatory drugs and antibiotics. Carbamazepine (CBZ) has been commonly implicated in SJS. Neuroleptic malignant syndrome (NMS) is a rare, life-threatening but potentially treatable condition. Among the neuroleptics, haloperidol (parenteral) is implicated as a most common drug for NMS. Though rare, association of NMS with CBZ and association of NMS with toxic epidermal necrolysis (TEN) in a single patient after administration of neuroleptics has been reported in the literature before. However, a combination of NMS and SJS in a single patient after administration of CBZ has not been reported so far. We present a patient with seizure who developed SJS and NMS following administration of CBZ.

Clinicodiagnostic features of tuberculous meningitis and the role of CSF PCR in early diagnosis: a study from north-west India.

The objective of this study was to study the clinical features and laboratory profile including acid fast bacillus microscopy, cerebrospinal fluid (CSF) culture and M. tuberculosis polymerase chain reaction (PCR) for an early diagnosis of tuberculous meningitis. Fifty consecutive patients, fulfilling the clinical criteria of tuberculous meningitis, were included in the study. All patients were subjected to detailed clinical and laboratory evaluation and radiological tests. CSF acid fast bacillus microscopy, culture and PCR were carried out in all and results were analysed. Majority of the patients presented with fever, headache, vomiting and signs of meningeal Irritation. Ziehl-Neelsen staining for acid-fast bacillus was positive in 4% cases, culture in 18% cases and CSF PCR for Mycobacterium tuberculosis was positive in 68%. Miliary pattern on chest x-ray was seen in 16% and CNS tuberculomas were seen in 14% cases. The CSF PCR estimation in clinically suspected cases of tuberculous meningitis may be an extremely valuable test for early diagnosis and institution of specific therapy.

Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report.

Neuropathic arthropathy of the shoulder is a relatively rare disorder characterized by destruction of joint secondary to loss of sensory innervation. Bilateral Charcot arthropathy is an even rarer disorder, with very few cases reported in the English literature. We herein present a case of bilateral shoulder arthropathy secondary to syringomyelia with classical clinical and radiological findings. Radiological finding on one side was of resorptive type and resorptive mixed with productive on the other side.

Reversible neurological syndromes with atypical pneumonia.

Simultaneous or sequential involvement of lungs is frequently encountered with neurological syndromes like meningoencephalitis, cerebellitis, aseptic meningitis, transverse myelitis, or multiple cranial nerve palsies. However, pulmonary involvement is frequently overlooked when all the attention of physician is diverted to neurological disorder. Prompt and early recognition of such potentially treatable association is required to improve diagnostic and therapeutic outcome. We report six patients presenting with various neurological manifestations like meningitis, meningoencephalitis, and myelits associated with atypical pneumonia. With proper clinical correlation and relevant investigations, all of them were diagnosed in time and had remarkable recovery with appropriate treatment.

Living longer living happier: My journey from clinical neurology to complexities of brain.

The present article is a treatise on the illuminating voyage of a Neurophysician along the fascinating horizons and frontiers of neurosciences. During the career as a clinical neurologist, some very interesting and intriguing cases and issues were dealt with and documented scientifically. The working of the brain and its operational architectonics came up for critical analysis, opening up new vistas in the appreciation and management of various neurological disorders. Issues regarding the working of the mind and the guidelines for health and happiness became apparent, and some very interesting generalizations with far-reaching consequences on the general well-being and health have been formulated and put forward for a healthy and happy future for mankind. A paradigm shift is warranted for a closer and better appreciation of neural dynamics at all levels of the brain, namely microscopic, mesoscopic and macroscopic levels!

Subarachnoid hemorrhage with transient ischemic attack: another masquerader in cerebral venous thrombosis.

Cerebral venous thrombosis has a wide spectrum of clinical manifestations that may mimic many other neurological disorders and lead to frequent misdiagnoses or delay in diagnosis. The most frequent symptoms and signs are headache, seizures, focal deficits, and papilledema. A number of rare atypical manifestations have been described. Cerebral venous thrombosis may present with an isolated intracranial hypertension type picture, thunderclap headache, attacks of migraine with aura, isolated psychiatric disturbances, pulsatile tinnitus, isolated or multiple cranial nerve involvement, and occasionally as subarachnoid hemorrhage (SAH) or transient ischemic attack. Our patient presented with thunderclap headache and transient ischemic attack like episode with obvious SAH on CT scan. Acute SAH suggests the presence of a vascular lesion, such as ruptured aneurysm, and CVT is not generally considered in the diagnostic workup of SAH. The case emphasizes the importance of cerebral venous study in nonaneurysmal cases of SAH. It is important to have a high index of suspicion in such atypical cases to avoid delay in diagnosis.

Neuromyotonia: clinical profile of twenty cases from northwest India.

OBJECTIVES: We are presenting 20 cases of the intriguing clinico-electromyographic entity, now considered a potassium channel disorder, Neuromyotonia. Our experience with the clinical manifestations, underlying abnormalities and response to various therapies is documented. MATERIALS AND METHODS: Patients with diffuse pain or undulating muscle movements, with or without stiffness were sent for electromyographic and further studies. Patients with "neuromyotonic discharges" were included after exclusion of hypocalcaemia. RESULTS: Our cases included 19 males and one female of age group 15 to 52 years, the majority being between 30 to 45 years. Undulating movements were seen in 19, of which two had focal twitching. Muscle stiffness was a complaint in five; pain was the chief presenting complaint of 19, which started in the calf in all. Irritability, insomnia and a peculiar worried pinched face were present in 12 patients. CSF was abnormal with mildly raised protein in eight. Curiously, 11 of these patients had taken ayurvedic treatment for various complaints in the preceding one month. Bell's palsy was associated in four, peripheral neuropathy in two and residual poliomyelitis in two. Electromyographic evidence of spontaneous activity in the form of "neuromyotonic discharges" was seen in all. Antibodies to voltage gated potassium channels was tested in one patient and was positive (titer was 1028 pM). Membrane stabilizers (e.g, phenytoin sodium) in our experience did not provide adequate rapid relief; we tried high-dose intravenous Methylprednisolone in 19 with significant amelioration of complaints. One patient was offered intravenous immunoglobulin, to which he responded. CONCLUSIONS: Neuromyotonia is a heterogeneous condition and can present in varied ways including diffuse nonspecific pain. This uncommon condition is potentially treatable and can be picked up with high index of suspicion.