A series of 18 cases of tuberculous encephalopathy (TBE) in children studied between June 1983 and October 1984 at Queen Elizabeth Hospital, Kota Kinabalu is presented. The data suggest that: the incidence is not as rare as previously thought; the occurrence of TBE is related to the absence of BCG vaccination; the presentation is often late; laboratory data, although helpful is not often confirmatory; early treatment is the key to better outcome; the clinical picture should outweigh laboratory diagnosis in the initial assessment and management.
The present study examines the gross anatomical features of anomalous lunate sulcus detected incidentally in a cadaveric brain and discusses its clinical importance. The absence of lunate sulcus was carefully studied in a dissected brain specimen. The absence of lunate sulcus was observed unilaterally on the right side of a cadaveric brain specimen. The lunate sulcus was clearly appreciated on the left side whilst on the right side it was absent. The right hemisphere of the cerebellum was also bigger in size as compared to the left. The absence of lunate sulcus is a rare finding, which may be detected incidentally. The anatomical knowledge of the lunate sulcus may be important for neurosurgeons operating on the occipital lobe and the radiologists interpreting CT scan.
Parkinsonism as a neurologic manifestation of dengue infection is rare with only 1 reported case in an adult patient. We report a case of a 6-year-old child with self-limiting post-dengue encephalopathy and Parkinsonism. This is the first reported pediatric case of post-dengue Parkinsonism and expands the neurologic manifestations associated with dengue infection in children. Clinicians should consider the possibility of post-dengue Parkinsonism in children with a history of pyrexia from endemic areas of dengue.
Colloid cysts are rare intracranial tumors most commonly found in the third ventricle. We present a case of colloid cyst of the third ventricle that manifested as bilateral advance optic disc cupping, superior hemifield defects in the visual fields and normal intraocular pressure.
: We present our initial experience using a simple and relatively cost effective system using existing mobile phone network services and conventional handphones with built in cameras to capture carefully selected images from hard copies of scan images and transferring these images from a hospital without neurosurgical services to a university hospital with tertiary neurosurgical service for consultation and management plan.
Heterotopic neuroglial tissue is a rare congenital lesion with predilection in head and neck region. We report a case of a newborn who presented with an oral cavity mass with intracranial extension and later respiratory distress that was successfully excised via transcranial and transcervical approach.
Cryptococcal infection of the brain as encountered in a tropical country is reviewed. The meningitic form is not uncommon and there has been, in the last decade, an apparent, if not real, rise in incidence in Malaysia as in Singapore. Only exceptionally was there overt evidence of immunological deficiency. Hydrocephalus was present in about three-quarters of the patients with meningitis and shunts were employed readily. The presence of multiple small intracerebral cysts could be suspected clinically but treatment for this complication was ineffective. The antifungal agent used most frequently was 5-fluorocytosine. Resistance to this drug developed in about one patient in four. There is a need for further epidemiological studies and for a continuing search for new antifungal agents.
Zinc is the authoritative metal which is present in our body, and reactive zinc metal is crucial for neuronal signaling and is largely distributed within presynaptic vesicles. Zinc also plays an important role in synaptic function. At cellular level, zinc is a modulator of synaptic activity and neuronal plasticity in both development and adulthood. Different importers and transporters are involved in zinc homeostasis. ZnT-3 is a main transporter involved in zinc homeostasis in the brain. It has been found that alterations in brain zinc status have been implicated in a wide range of neurological disorders including impaired brain development and many neurodegenerative disorders such as Alzheimer's disease, and mood disorders including depression, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and prion disease. Furthermore, zinc has also been implicated in neuronal damage associated with traumatic brain injury, stroke, and seizure. Understanding the mechanisms that control brain zinc homeostasis is thus critical to the development of preventive and treatment strategies for these and other neurological disorders.
Recent progress in genetic analysis reveals that a significant proportion of cryptogenic epileptic encephalopathies are single-gene disorders. Mutations in numerous genes for early-onset epileptic encephalopathies have been rapidly identified, including in SPTAN1, which encodes α-II spectrin. The aim of this review is to delineate SPTAN1 encephalopathy as a distinct clinical syndrome. To date, a total of seven epileptic patients with four different in-frame SPTAN1 mutations have been identified. The major clinical features of SPTAN1 mutations include epileptic encephalopathy with hypsarrhythmia, no visual attention, acquired microcephaly, spastic quadriplegia and severe intellectual disability. Brainstem and cerebellar atrophy and cerebral hypomyelination, as observed by magnetic resonance imaging, are specific hallmarks of this condition. A milder variant is characterized by generalized epilepsy with pontocerebellar atrophy. Only in-frame SPTAN1 mutations in the last two spectrin repeats in the C-terminal region lead to dominant negative effects and these specific phenotypes. The last two spectrin repeats are required for α/β spectrin heterodimer associations and the mutations can alter heterodimer formation between the two spectrins. From these data we suggest that SPTAN1 encephalopathy is a distinct clinical syndrome owing to specific SPTAN1 mutations. It is important that this syndrome is recognized by pediatric neurologists to enable proper diagnostic work-up for patients.
Video-EEG telemetry is often used to support the diagnosis of non-epileptic seizures (NES). Although rare, some patients may have both epileptic seizures (ES) and NES. It is crucially important to identify such patients to avoid the hazards of inappropriate anticonvulsant withdrawal. To delineate the electroclinical characteristics and diagnostic problems in this group of patients, we studied the clinical, EEG and MRI features of 14 consecutive patients in whom separate attacks, considered to be both NES and ES were recorded using video-EEG telemetry. Only two patients were drug-reduced during the telemetry. Most patients had their first seizure (ES or NES) in childhood (median age 7 years; range: 6 months-24 years); 8/14 patients were female. Brain MRI was abnormal in 10/14 patients. Interictal EEG abnormalities were present in all patients; 13/14 had epileptiform and 1/14 only background abnormalities. Over 70 seizures were recorded in these 14 patients: in 12/14 patients, the first recorded seizure was a NES (p < 0.001), and 7 of these patients had at least one more NES before an ES was recorded. Only 3/14 patients had more than 5 NES before an ES was recorded. Recording a small number of apparently NES in an individual by no means precludes the possibility of additional epilepsy. Particular care should be taken, and multiple (> 5) seizure recording may be advisable, in patients with a young age of seizure onset, interictal EEG abnormalities, or a clear, potential aetiology for epilepsy.
A prospective study was carried out to determine the incidence, clinical presentation, early outcome, and risk factors associated with periventricular haemorrhage (PVH) in 88 (84 per cent) of the 105 consecutive very low birth weight (VLBW) (< 1500 g) Malaysian neonates born in the Maternity Hospital, Kuala Lumpur. Based on the cranial ultrasound findings, PVH was detected in 86 of the 88 neonates (98 per cent, 95 per cent confidence intervals: 95 to 101). Seventeen (20 per cent) of them had grade I, 52 (61 per cent) had grade II, 7 (8 per cent) had grade III and 10 (12 per cent) had grade IV PVH. PVH was detected in all the affected neonates by the fifth day of life. Sixty-four neonates (74 per cent) were symptomatic when PVH was first detected. Shock (P < 0.01), pallor (P = 0.028), low haematocrit of less than 40 per cent (P < 0.01), convulsion (P < 0.001), and bulging of anterior fontanelle (P = 0.019) were significantly more common in the neonates with severe PVH (grades III or IV). Death occurred in 43/86 (50 per cent, 95 per cent confidence interval: 39-61 per cent) of the neonates with PVH before their first discharge from the hospital. Ventriculomegaly developed in 29/43 (67 per cent, 95 per cent confidence intervals: 54.4-81.4) of the survivors with PVH. Our study suggests that PVH is a common problem in the Malaysian VLBW neonates. To reduce the incidence and severity of this condition, prevention of preterm delivery and improvement in the basic facilities for neonatal care would help.
Patients with end stage renal disease have higher risk of tuberculosis due to lower cell-mediated immunity. Standard regime of anti-tuberculosis contains isoniazid where neurological side effects such as seizure and encephalopathy have been documented. We present a case of isoniazid-induced encephalopathy in a haemodialysis patient. A literature review on isoniazid-induced encephalopathy was done. Recognition of this condition is important as it is reversible with cessation of isoniazid and institution of high dose pyridoxine.
Rhino-orbito-cerebral mucormycosis is a fungal infection that can be fatal especially in immunocompromised patients. It is extremely rare in immunocompetent individuals. We describe here an immunocompetent patient who survived rhino-orbito-cerebral mucormycosis due to Saksenaea vasiformis, and provide a literature review of this rare entity.