Pradeep Pankajakshan Nair

Semiologic classification of psychogenic non epileptic seizures (PNES) based on video EEG analysis: Do we need new classification systems?

PURPOSE Different studies have described useful signs to diagnose psychogenic non-epileptic seizure (PNES). A few authors have tried to describe the semiologic groups among PNES patients; each group consisting of combination of features. But there is no uniformity of nomenclature among these studies. Our aim was to find out whether the objective classification system proposed by Hubsch et al. was useful and adequate to classify PNES patient population from South India. METHODS We retrospectively analyzed medical records and video EEG monitoring data of patients, recorded during 3 year period from June 2010 to July 2013. We observed the semiologic features of each PNES episode and tried to group them strictly adhering to Hubsch et al. classification. Minor modifications were made to include patients who were left unclassified. RESULTS A total of 65 patients were diagnosed to have PNES during this period, out of which 11 patients were excluded due to inadequate data. We could classify 42(77.77%) patients without modifying the defining criteria of the Hubsch et al. groups. With minor modification we could classify 94.96% patients. The modified groups with patient distribution are as follows: Class 1--dystonic attacks with primitive gestural activities [3(5.6%)]. Class 2 – paucikinetic attacks with or without preserved responsiveness [5(9.3%)]. Class 3--pseudosyncope with or without hyperventilation [21(38.9%)]. Class 4--hyperkinetic prolonged attacks with hyperventilation, involvement of limbs and/or trunk [14(25.9%)]. Class 5--axial dystonic attacks [8(14.8%)]. Class 6--unclassified type [3(5.6%)]. CONCLUSION This study demonstrates that the Hubschs classification with minor modifications is useful and adequate to classify PNES patients from South India.

Left-sided Poland's syndrome in a girl with rare associations like spina bifida and diaphragmatic hernia

Polands syndrome is a rare congenital anomaly characterised by partial or complete absence of sternocostal head of pectoralis major muscle and anomalies of ipsilateral hand and digits. Other associated anomalies involving anterior thoracic wall, breast, diaphragm and vertebrae have also been reported in various cases. We report a case of a 10-year-old girl, with features of left-sided Polands syndrome associated with spina bifida, dextroposition of the heart and left-sided diaphragmatic hernia. These are rare associations of Polands syndrome. She was investigated with chest X-ray, contrast-enhanced CT of the thorax, ultrasonography of abdomen and echocardiography which helped in arriving at an accurate diagnosis and assessing all the associated abnormalities.

Refractory status epilepticus complicated by drug-induced involuntary movements

New onset refractory status epilepticus (NORSE) is a neurological emergency and difficult to treat condition. We report a case of involuntary movements resulting from thiopentone sodium infusion during the management of refractory status epilepticus. A young woman was admitted with fever and NORSE in the neurology intensive care unit. In addition to supportive measures, she was treated with intravenous lorazepam, phenytoin sodium, sodium valproate, midazolam and thiopentone sodium. While on thiopentone sodium, she developed involuntary twitches involving her upper limbs and face with EEG showing no evidence of ongoing status epilepticus. Because of the temporal relationship with thiopentone infusion, we tapered the dose of thiopentone sodium, which resulted in the disappearance of the movements. The patient recovered well with no recurrence of the seizures during the hospital stay.

Teaching Neuroimages: Snake eyes appearance in MRI in patient with ALS

A 17-year-old girl presented with 6 months of progressive asymmetrical distal weakness of upper limbs with fasciculation. There were no bulbar symptoms, weakness in lower limbs, or sensory symptoms. There was no history of neck injury or pain. Deep tendon reflexes were all brisk. MRI of cervical spine revealed symmetrical T1 hypointense, T2 hyperintense signal in the anterior horns (“snake eyes” appearance) (figure, A and B) without evidence of extradural compression. EMG showed diffuse denervation changes in both upper and lower limb muscles. Sensory nerve action potentials were normal. A diagnosis of amyotrophic lateral sclerosis (ALS) was made. Snake eyes appearance has been described in disorders like ALS, cervical spondylotic amyotrophy, Hirayama disease, and ossification of posterior longitudinal ligament.1,2

Sporadic Creutzfeldt-Jakob disease (sCJD) with asymmetric findings

We report a case of a patient with probable Creutzfeldt-Jakob disease (CJD) who had psychiatric manifestation in the form of withdrawn depressive behaviour at the onset, followed by rapidly progressive ataxia, parkinsonism, mutism and cognitive decline with generalised asynchronous multifocal myoclonic jerks. His EEG exhibited focal (lateralised) periodic triphasic sharp waves on the background of generalised delta slowing, which later on became more generalised. MRI of the brain showed hyperintensity in basal ganglia with cortical ribbon sign in bilateral frontal region. Clinical course showed progressive deterioration to an akinetic-abulic stage. He died 2 months after the onset of symptoms.

Transient bradycardia induced by thiopentone sodium: a unique challenge in the management of refractory status epilepticus.

Thiopentone sodium is one of the important drugs in the armamentarium for terminating refractory status epilepticus, a neurological emergency. We report a case of thiopentone-related bradycardia during the management of the new onset refractory status epilepticus in a young man, which was circumvented by prophylactic insertion of temporary pacemaker while thiopentone infusion was continued. A systematic approach was employed to manage the status epilepticus, including infusion of thiamine and glucose followed by antiepileptic drugs. The patient was ventilated and infused with lorazepam, phenytoin, sodium valproate, levetiracetam and midazolam followed by thiopentone sodium. With the introduction of thiopentone the seizures could be controlled but the patient developed severe bradycardia and junctional rhythm. The bradycardia disappeared when thiopentone was withdrawn and reappeared when the drug was reintroduced. Propofol infusion was tried with no respite in seizures. Later thiopentone sodium was reintroduced after inserting temporary cardiac pacemaker. Seizure was controlled and patient was weaned off the ventilator.

Acute bilateral putaminal haemorrhagic necrosis in methanol poisoning

A 30-year-old man without significant past illness was admitted to the neurology intensive care unit with a 2-day history of altered level of consciousness, vomiting and vision loss. History revealed that he had consumed unknown amounts of unbranded alcohol 4 days earlier. The clinical examination revealed a Glasgow Coma Scale of E2V2M4, bilaterally dilated pupils, optic disc oedema, normal deep tendon reflexes and flexor plantar responses bilaterally. Arterial blood gas analysis showed severe metabolic acidosis (pH 7.11). Further evaluation revealed high …

Teaching NeuroImages: Neuropathic elbow arthropathy due to syringomyelia

A 45-year-old woman presented with 3 months of left elbow swelling and minimal pain ([figure][1], A). She did not remember any upper extremity injury. On examination, she had arthritis of left elbow, wasting of small muscles of the hand, depressed biceps and supinator jerks, and decreased pain and

New-onset interictal headache in Persons with epilepsy (PWE): A case–control study

PURPOSE In our personal experience, headache is a frequent complaint in Persons with epilepsy (PWE) receiving antiepileptic drugs (AEDs), carbamazepine (CBZ) in particular. There is hardly any systematic study on AEDs-associated headache. We assessed the association of new-onset interictal headache in PWE with CBZ compared with other AEDs and factors associated with it. METHODS Two thousand PWE on AEDs were screened, and 165 persons having new-onset interictal headache were identified. Eight persons were excluded because of comorbid illnesses that could potentially contribute to a headache. Finally, 157 were recruited for the study. One hundred fifty-seven age- and sex-matched PWE on AEDs, without headache, were recruited as controls. Headache questionnaire based on the International Classification of Headache Disorders (ICHD-II) criteria was used. Demographic factors, seizure characteristics, type and dose of AEDs, findings on electroencephalogram (EEG), and magnetic resonance imaging (MRI)/computerized tomography (CT) were recorded. RESULTS Among PWE with headache, the majority [88 (56.1%)] were on CBZ compared with other AEDs but not significantly different from controls. In 71 (45%) persons, the headache had migrainous character. In both univariate and multivariate analyses, lower seizure frequency and fewer EEG abnormalities were found to be independent predictors of headache. CONCLUSIONS New-onset interictal headache is a significant issue in PWE; nonmigrainous headache is more common than the migrainous type. Carbamazepine was not found to have any predilection for new-onset interictal headache compared with other AEDs. A lower frequency of seizures and EEG abnormalities in PWE with headache may suggest that seizures contribute less to the development of headache and may further support the hypothesis that AEDs may be significant contributing factors for the headache.

Intradiploic cephalocele: a rare entity at a rare site

A 52-year-old woman presented with complaints of non-specific headache. The patient had a history of head injury 1 year ago. Clinical neurological examination was nil significant. The skin over the occipital region is normal. No prior investigations were done immediately after the injury. MRI of the brain at present showed small intradiploic cerebrospinal fluid (CSF)-filled defect with herniation of foliae of the right cerebellar hemisphere (figure 1). The rest of the brain parenchyma revealed no abnormality and no skull fracture …