Hypereosinophilia-an unusual cause of multiple embolic strokes and multi-organ dysfunction.

'Eosinophilia' can occur due to a large number of allergic, infectious, neoplastic, and idiopathic diseases. It can range in severity from a self-limiting condition to a life-threatening disorder. The term 'hypereosinophilia' refers to eosinophil levels >1500/µL, and regardless of the underlying cause can be associated with tissue and organ damage. 'Hypereosinophilic syndrome (HES)' is a rare disorder with sustained eosinophilia and multi-organ dysfunction in the absence of a discernable secondary cause. 'Undefined Hypereosinophilic Syndrome' is the most common type of primary hypereosinophilic diseases and we are reporting here one such case who presented with acute multiple embolic strokes secondary to biventricular apical thrombi and multi-organ dysfunction of a fulminant nature. This case highlights the limitation in current diagnostic criteria for HES and emphasizes the need for early intervention.

Recurrent prolonged fugue states as the sole manifestation of epileptic seizures.

A fugue state is defined as an altered state of consciousness with varying degrees of motor activity and amnesia for the event. It may last for hours to days and may be psychogenic or organic in nature. Epileptic fugue states can be encountered in patients with absence or complex partial nonconvulsive status epilepticus or may occur as a postictal phenomenon in patients with generalized seizures. "absence status epilepticus" (AS) is rare and seen in only 2.6% of the cases with "childhood absence epilepsy" (CAE). The diagnosis of AS can be elusive, but sudden onset and termination of the fugue state, classical electroencephalogram (EEG) features, and response to a therapeutic trial of benzodiazepines helps in confirming the diagnosis and differentiating it from nonepileptic fugue states. We report a childhood onset case, with a 10 years history of recurrent episodes of prolonged fugue state lasting for up to 24 h, as the sole manifestation of epileptic seizures. The EEG features were suggestive of an AS, but there was no history of typical absences, myoclonus, or generalized tonic clonic seizures. This unusual and rare case cannot be categorized into one of the defined epilepsy syndromes like CAE but belongs to a recently identified syndrome of idiopathic generalized epilepsy known as "Absence status epilepsy" in which AS is the sole or the predominant seizure type.

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.

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.

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.

Pathological laughter associated with paroxysmal kinesigenic dyskinesia: A rare presentation of acute disseminated encephalomyelitis.

A 13-year-old boy presented with recurrent episodes of sudden brief posturing of the right upper and lower limbs accompanied by transient inability to speak and a tendency to smile which would sometimes break into laughter. Awareness was retained during the attack, and there was no associated emotional abnormality. The events were precipitated by walking and occurred several times in a day. The laughter was pathological in nature, and the abnormal posturing was akin to 'paroxysmal kinesigenic dyskinesia' (PKD). 'Pathological laughter or crying' is defined as an involuntary, inappropriate, unmotivated laughter, crying or both, without any associated mood change. It can occur as a result of cerebral lesions like tumors, trauma, vascular insults, multiple sclerosis and/or degenerative disorders. It can also be a component of gelastic epilepsy which is characterized by stereotyped recurrences, presence of interictal and ictal epileptiform discharges and absence of external precipitants. In our patient, however, there was no ictal or interictal EEG correlate. Paroxysmal kinesigenic dyskinesia is characterized by intermittent, involuntary movements triggered by kinesigenic stimuli and is usually familial but can also be secondary to metabolic and structural brain disorders. Magnetic Resonance Imaging (MRI), in our case, revealed multiple T2 and FLAIR hyperintense, non-enhancing lesions in the periaqueductal gray matter, pontine and midbrain tegmentum, bilateral thalami and left lentiform nucleus suggesting a diagnosis of 'acute disseminated encephalomyelitis', in which this unique combination of pathological laughter and PKD has not been described so far. Magnetic Resonance Spectroscopy (MRS) confirmed a demyelinating pathology, and the patient responded well to steroids.

Restless leg syndrome, periodic limb movements, febrile seizures and Attention deficit hyperactivity disorder in an Indian family.

Restless leg syndrome (RLS) is a common neurological disorder which can affect individuals of all age groups and incidence increasing with age. It can cause severe sleep disruption and negatively impact quality of life of an individual. Its diagnosis is clinical, based on essential criteria of International RLS Study Group. It can be idiopathic or associated with various medical and other neurological disorders. Idiopathic RLS can be sporadic or may have a familial inheritance, with several genetic loci been reported till date. RLS has a strong association with periodic limb movements, both sleep and awake. Very few studies of familial RLS/Periodic limb movements in sleep and their associations have been reported. We report an Indian family with autosomal dominant RLS/PLMS, with RLS and PLMS as well as psychiatric disorders, febrile seizures and Attention Deficit Hyperactivity Disorder in different family members, over three generations.

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.

Hallucinations in Parkinson's disease--a study of forty three patients.

OBJECTIVE: To see the prevalence of hallucinations in patients with Parkinson's disease and to compare the various parameters between hallucinators and non-hallucinators. METHODS: Forty-three patients of Parkinson's disease were included. Detailed motor assessment was done with UPDRS scales. Assessment was done for the presence of depression and sleep disturbances. The patients were enquired for the presence of depression and sleep disturbance. The patients were enquired for the presence of hallucinations. Hallucination types and associated emotional experience were probed into. Comparative analysis was subsequently done between hallucinators and non-hallucinators. RESULTS: The mean age of the patients was 61.47 years while mean duration of symptoms of PD was 4.30 years. The mean UPDRS II and III scores were 15.18 and 38, respectively. Fifteen patients (34.9%) had experienced hallucinations. The hallucinations described were visual as well as auditory in nature. Majority of these patients (12 out of 15, 80%) had not volunteered about their hallucinations on their own. On analysis of various parameters between the hallucinators and non-hallucinators, it was observed that hallucinators were older and had a higher mean duration of symptoms of PD. The patients with hallucinations had a higher severity of motor symptoms and signs. Hallucinators more commonly had depression and sleep disturbances. Mean dosage of L-dopa equivalent dose was higher in patients with hallucinations as compared to those without hallucinations. On statistical analysis, however, only two parameters were found to be different in a statistically significant manner. These were the duration of illness and the Hoen and Yahr scale (p < 0.05). Also, hallucinations occurred independent of dopaminergic drugs. CONCLUSION: Hallucinations are common source of distress but are often neglected. One should always probe actively into the presence of hallucinations.