Lokesh Saini

Finger drop sign: Rare presentation of a common disorder.

BACKGROUND Guillain Barre syndrome (GBS) commonly presents with limb weakness and occasional cranial nerve, respiratory or autonomic involvement. Isolated or predominant bilateral finger drop as presenting feature has never been reported in the pediatric age group. CASE A 9-year-old boy presented with deformity of both hands for 7 days and leg pain with difficulty in getting up from floor for 3 days. On examination he had bilateral clawing with subtle hip flexor weakness and hyporeflexia. His nerve conduction study revealed motor axonal neuropathy. His serum lead levels and autoimmune markers were within normal limits. His cerebrospinal fluid examination revealed albuminocytological dissociation. He was diagnosed as GBS and was given intravenous immunoglobulin. He improved completely over next 8 weeks. CONCLUSIONS GBS is one of the commonest causes of acquired neuropathy in the tropics. In resource limited setting, where electrophysiological facilities may not be available, identification of finger drop sign may help in correct management.

Cystic lesion of the fourth ventricle Role of CISS

A 13-year-old boy presented with headache of 1 month and vomiting for 10 days. Salient examination findings were left lateral rectus paresis and papilledema. A contrast-enhanced CT followed by MRI brain revealed an ill-defined fourth ventricular cyst with communicating hydrocephalus (figure 1). Subsequently, a constructive interference in steady state (CISS) sequence revealed a fourth ventricular cysticercus (figure 2).

Health Related Quality of Life in Adult Obstructive Sleep Apnea

Objective: To review the literature on Obstructive Sleep Apnea (OSA) and Health Related Quality of Life (HRQoL). Method: We conducted systematic review of the literature on HRQoL and OSA. Summary: Our review of published literature on quality of life in adult OSA suggest that there is significant impairment of HRQoL in obstructive sleep apnea but the magnitude of QoL impairment is not proportional to the severity of OSA as determined by AHI. There is paucity of data over effect of treatment on quality of life but the available data suggest that there is improvement in QoL with CPAP treatment at least in moderate and severe OSA patients. Effect of other treatment modalities is unclear yet.

Recurrent headache in a five year old boy

Headache is infrequent in early childhood. Headache and neurological deficits associated with cerebrospinal fluid (CSF) lymphocytosis (HaNDL), a variant of migraine, is a rare disorder. A 5-year-old boy presented with recurrent episodes of headache for 6 months. Each episode lasted for a week and in the current episode, he was symptomatic for 3 days. All the episodes were associated with paresthesias and CSF lymplocytosis with normal protein and sugar. There was history of migraine in his family. His magnetic resonance imaging (MRI) brain with contrast with magnetic resonance (MR) angiography and venography were normal. Work-up for relevant causes of infection and vasculitis were negative. His symptoms subsided on oral antimigraine prophylaxis and he has been on remission for last 8 months. HaNDL should be considered in relevant clinical scenarios, as it prevents unnecessary investigations, therapy, and hospitalization.

A Combination of Moyamoya Pattern and Cerebral Venous Sinus Thrombosis: A Case of Tubercular Vasculopathy

BACKGROUND Moyamoya vasculopathy, arising secondary to tubercular meningitis (TBM) is unusual. There have also been a few reports of cerebral venous sinus thrombosis (CVST) in TBM. A case of TBM, complicated simultaneously by Moyamoya syndrome and CVST, is being presented here. CASE A 1-year-old girl presented with febrile encephalopathy, vomiting, seizures and left hemiparesis. Cerebrospinal fluid analysis was suggestive of TBM. Extensive infarcts were noted in the magnetic resonance imaging, involving right middle cerebral artery (MCA), anterior cerebral artery and the left MCA. Magnetic resonance venogram revealed left transverse venous sinus thrombosis and magnetic resonance angiography showed bilateral moyamoya pattern of arteriopathy. Patient was started on antitubercular therapy and low molecular weight heparin. CONCLUSIONS Early vascular involvement affecting both arterial and venous structures has not hitherto been reported in CNS tuberculosis. Early recognition of secondary complications of CNS tuberculosis is crucial to prevent the morbidity and mortality associated with TBM.

Unexplained Transaminitis and Hyperactivity: Do Not Forget the Musculoskeletal Examination

To the Editor: An eleven-year old boy presented with recurrent abdominal pain, hyperactivity and aggressive behavior. His anthropometry, general and systemic evaluation were within normal limits. His investigations (prior to referral) revealed transaminitis (alanine aminotransferase of 2021 U/L and aspartate aminotransferase of 931 U/L). Without further thought and this symptom complex, Wilson’s disease was initially considered. His evaluation for chronic liver disease (viral hepatitis, ceruloplasmin, serum copper, autoimmune antibodies, MRI brain, slit lamp for Kayser–Fleischer rings) were unremarkable. The child was referred to our center to find out the etiology of this unexplained transaminitis. A review history revealed difficulty in running and examination revealed pseudohypertrophy of calf and deltoid (Fig. 1) and mild proximal weakness. With a clinical possibility of Becker’s muscle dystrophy (BMD), creatine phosphokinase (CPK) was evaluated and was elevated 6747 U/L. 79-exon multiplex PCR of dystrophin gene confirmed deletion of exon 2 and 5, confirming the diagnosis of BMD. The case is important as it highlights the dwindling art of the clinical examination. It is not uncommon to see that with ease of availability of investigations, the physicians tend to get lost in the clinical diagnosis even before comprehensive clinical assessment. Had the clinical examination been complete, it would not have taken longer to recognize that transaminitis could be of muscle origin. Transaminitis is common in muscle disease, seen in 97.5% cases with Duchene muscular dystrophy (DMD) and all cases of BMD. The degree (median) of transaminitis is more in DMD compared to BMD (6.55 times vs. 2.91 in BMD). Mild transaminitis of 1.65 times may also be seen in limb girdle dystrophy [1]. So, to a certain extent, degree of transaminitis may help in differentiating muscle dystrophies. It is not uncommon to see such children being subjected to liver biopsies [2]. In one series, 36% and 32% children of BMD had attention deficit hyperkinetic disorder and learning disabilities [3]. So, in a child with behavioral abnormalities and/or learning difficulties, who has physical difficulties in coping with other children, a possibility of muscle disease should be evaluated. We suggest that all children with unexplained transaminitis should be asked history of proximal muscle weakness symptoms and evaluated clinically for pseudohypertrophy and subsequently by CPK-total which may save exorbitant cumbersome tests for liver disease. * Lokesh Saini drlokeshsaini@gmail.com

Recurrent Cerebrospinal Fluid Pleocytosis

To the Editor: An 11 y boy presented with severe continuous headache for the last 72 h. He has history of recurrent headaches for the last 2 y. Headache would occu r 3–5 t imes pe r mon th and l a s t 1–3 h . Intermittently these were associated with photophobia, phonophobia, vomiting, retro-orbital pain and transient paresthesias of hand. Cerebrospinal fluid (CSF) evaluat ion had shown lymphocyt ic pleocytosis (Table 1). He had been treated with antibiotics for meningitis during the first two episodes. Detailed evaluation for chronic meningitis (tuberculosis, cryptococcus meningitis, herpes polymerase chain reaction, cytomegalovirus, acanthamoeba, fungal culture, HIV ELISA and malignant cytology) was unremarkable. Contrast magnetic resonance imaging, angiography and venography were normal. He was managed as a case of headache and neurological deficits with CSF lymphocytosis (HaNDL). He was treated with anti-emetics and oral rizatriptian (no antibiotics) for the acute episode, and subsequently started on flunarizine prophylaxis. The child is asymptomatic for the last 6 mo. The case aims to sensitize the pediatricians about non-infectious etiology of CSF pleocytosis in a child with severe headache. Acute headaches in children can be due to sinusitis, migraine, meningitis, intracranial hemorrhage or brain tumors [1]. A normal neuroimaging excludes hemorrhage and tumors, however the clinical distinction between the other etiologies can be challenging. The location of pain and character of pain in sinusitis, migraine and meningitis is commonly fronto-temporal and squeezing or pounding in nature [1]. Due to its seriousness, cerebrospinal fluid analysis is usually done as in the index case to exclude meningitis. Since all the evaluations showed pleocytosis, a possibility of recurrent and chronic meningitis was suspected. However, fever was notably absent during all the episodes and infective markers like C-reactive protein (CRP) and procalcitonin were repeatedly negative. This led to a suspicion of non-infectious etiology like HaNDL which is characterized by a prodrome of viral illness followed by severe headache (lasting 1 h-1 wk) and neurological deficits [2]. The deficits in the index case were only in form of paresthesias which may be due to sluggish blood circulation in the corresponding area of cortex [3]. To conclude, the knowledge of this syndrome is important to limit unnecessary evaluation (multiple costly invasive and noninvasive investigations) and treatment of chronic meningitis with multiple antibiotics in a child with recurrent headaches associated with CSF pleocytosis.

Levetiracetam Induced Neuropsychiatric Manifestation in a 5-year-old Boy

To the Editor: A five-year-old developmentally normal boy presentedwith six episodes of seizures (Generalized tonic-clonic) for last 6 mo. He was initially started on phenytoin but later switched over to levetiracetam (30mg/kg) because of poor seizure control. Two weeks after starting of levetiracetam, the child had a change in behavior like poor attention, poor concentration span and decreased interaction with others. Three weeks later, he had intermittent episodes of crying and shouting and hitting other family members without any reason. He also had suicidal tendencies in the form of attempts to cut his wrist with a knife and selfstrangulation with a belt. Evaluation for vasculitis (complete blood counts and anti-nuclear antibody), autoimmune encephalitis, worsening epilepsy and other structural etiologies were unremarkable (normal awake and sleep electroencephalography and MRI brain with MR-angiography). Clinical diagnosis of genetic generalised epilepsy was considered. A possibility of levetiracetam induced neuropsychiatric side-effects was considered and levetiracetam was switched over to topiramate. Within few days, he had improvement in interaction with parents, sleep, and behavior. Improvement in symptoms following the withdrawal of levetiracetam suggested a probable causality. He was discharged after 1 wk, and he remained symptom-free over the next 3 mo. Behavioral and cognitive impairments are common in children with epilepsy. These symptoms can be epilepsy itself or due to treatment emergent side-effects of anti-epileptic drugs. Twenty to 30 % of patients with epilepsy have associated mood disorders, and 2–7% may have frank psychosis [1]. Behavioral adverse effects are common with levetiracetam and are seen in 5– 34% of children [2]. Most of these are mild and seldom need discontinuation of levetiracetam. Common adverse effects include aggression, excessive sleepiness, hyperactivity and depression [2, 3]. Rapid titration and prior intellectual/learning disability were associated with higher incidence of adverse effects. Severe adverse effects like severe depression, psychosis, and suicidal tendencies are rare [4, 5]. The suicidal tendencies may be explained by bouts of rage and aggression in the index child, which may lead to self-harm. Concomitant use of pyridoxine has been described to ameliorate behavioral side-effects of levetiracetam by decreasing hyperactivity. To conclude, the possibility of severe behavioral abnormalities should be borne in mind even with commonly used and seemingly safe antiepileptic drugs.

Acute Flaccid Paralysis: Intravenous Immunoglobulin is Not the Drug of Choice Always!

To the Editor: A 7-y-old boy presented with a history of pain in the limbs for 10 d, which was characteristically more during the evening and night, and weakness of all 4 limbs for 5 d. There was no history of altered sensorium, seizures, preceding episode of loose stools or upper respiratory tract infection, trauma, tick bite, dog bite or rash. On examination, he had normal mentation and right lower motor neuron facial nerve palsy. His power in right upper and both lower limbs were 3/5 and in left upper limb, it was 4/5 at all the joints. Muscle stretch reflexes were absent. Sensory and cerebellar examination was normal. He had neck stiffness and positive Kernig’s sign. Clinical diagnosis of meningoradiculitis and atypical presentation of Guillian Barré syndrome was made. Nerve conduction revealed motor axonal polyneuropathy. MRI whole spine revealed post contrast enhancement of dorsal and ventral roots suggestive of radiculitis (Fig. 1a-e]. Cerebrospinal fluid (CSF) examination showed pleocytosis (80 cells/mm, 100% lymphocytes), elevated protein (105 mg/dl) and normal sugar (45.6 mg/dl). CSF culture was sterile. CSF picture compelled us to send for Lyme’s and HIV serology; HIV was non-reactive, however Lyme’s serology was strongly positive (Serum IgM, IgG antibody levels were 40.79 aU/ml, 7.37aU/ml respectively). A diagnosis of Lyme’s meningoradiculitis was concluded. The child was started on intravenous ceftriaxone for two weeks. After 2 wk, power was 4/5 in all the 4 limbs and there were no meningeal signs. Neurological manifestations (Neuroborreliosis) is seen in about 10% of patients with Lyme disease and it includes cranial neuropathy, mononeuritis multiplex, radiculitis and lymphocytic meningitis. Index child was fulfilling the criteria for Lyme’s meningoradiculitis, which in fact is the most frequent manifestation of early disseminated Lyme’s disease [1]. In our patient we could not test CSF antibodies to Lyme’s in view of economic constraints. Some patients can reveal negative serology in their serum [2, 3] or even no CSF synthesis of specific immunoglobulins [4]; however absence of both does not necessarily rule out Lyme meningoradiculitis [2–4]. The diagnosis of Lyme meningoradiculitis should be suspected in all patients with suspicion of GBS who are residing in endemic regions and present with radicular pains preceding the weakness, predominant signs of meningeal irritation, unilateral or bilateral facial nerve involvement with or without a lymphocytic CSF picture as therapy is different and rewarding and if picked early, can prevent significant neuromorbidity and unnecessary costly therapies like immunoglobulins.

Comparison of telephone with face to face consultation for follow up of Neurocysticercosis

OBJECTIVES There is significant scarcity of specialists to provide care for children with epilepsy in many parts of the world. Telemedicine is a potential future option. This study was planned to estimate the diagnostic accuracy of telephone consultation to identify Critical Clinical Events (breakthrough seizures, drug non-compliance, drug adverse events, features of raised intracranial pressure, and other disease-related events),compared to the Face-to-Face consultation (gold standard), in children with Neurocysticercosis (NCC) and symptomatic seizures, following the completion of cysticidal therapy. METHODS Children aged 2-15 years attending a tertiary health care facility with a diagnosis of NCC and symptomatic seizures were enrolled after completion of the cysticidal therapy. The parents were contacted by a Pediatric Neurology Resident on Telephone before the scheduled hospital visit. Subsequently, all the children were seen directly in hospital the next day by another Pediatric Neurology Resident. The information was noted on a structured questionnaire. The diagnostic accuracy of telephone consultation for identifying the Critical Clinical Events was estimated using Face-to-Face consultation as the gold standard. RESULTS A total of 1145 potential events were evaluated. Of these, the face-to-face consultation identified 56 events that would need hospital visit for detailed evaluation (breakthrough seizures in 19, drug non-compliance in 15, adverse drug events in 11, features of raised intracranial pressure in 8, and other disease-related events in 3), and 1089 events that did not require hospital consultation. The sensitivity, specificity, positive and negative predictive values of telephone consultation were 89.28% (78.12-95.96), 97.61% (96.52-98.43), 65.79% (54.01-76.30), and 99.43% (98.78-99.79) respectively. The likelihood ratios when telephone consultation was positive and negative were 37.3 and 0.11 respectively. SIGNIFICANCE Telephone consultation is an acceptable mode of follow-up for children with mild Neurocysticercosis and symptomatic seizures after completion of cysticidal therapy.