Decompressive hemicraniectomy for malignant middle cerebral artery infarction. Experience from the Western Province of Saudi Arabia.

OBJECTIVE: To describe our experience implementing decompressive hemicraniectomy (DH) for eligible patients with malignant middle cerebral artery (MCA) infarcts. METHODS: We retrospectively collected data of malignant MCA infarction patients requiring DH at King Abdulaziz University Hospital & King Faisal Specialist Hospital & Research Center, Jeddah, Kingdom of Saudi Arabia between October 2010 and July 2015. Clinical outcome was assessed immediately postoperatively using Glasgow Coma Score (GCS), and at 12 months using the modified Rankin scale (mRS) and Barthel index. Survival was evaluated at thirty-days and one year after surgery. RESULTS: Six out of 10 patients diagnosed with malignant MCA infarction underwent DH. Among the surgically treated patients (n=6), 4 were males (66%), and the median age was 22.5 years. The median time from admission to surgery was 35.5 hours. The median post-operative GCS was 6.5. Three patients (50%) died within 30 days of DH. In those who survived, the median mRS was 4.5 and BI was 7.5. CONCLUSION: Decompressive hemicraniectomy saves life and has the potential of improving survival functional outcome when done fast and in carefully selected patients. We call for national awareness of the management of such cases and early intervention.

Severe proximal myopathy with remarkable recovery after vitamin D treatment.

BACKGROUND: Osteomalacia is an uncommon cause of muscle weakness. Our objectives were to describe features of myopathy associated with Vitamin D deficiency and examine the contributing factors leading to osteomalacic myopathy in our region. METHODS: Patients identified retrospectively for the six year period ending in December 2006 with the diagnosis of osteomalacia and/or Vitamin D deficiency associated proximal muscle weakness were included. They were followed in three major centers in western Saudi Arabia. Clinical, biochemical, radiological, and electrophysiological findings were collected before and after Vitamin D treatment by chart review. RESULTS: Forty seven female patients aged 13-46 years (mean 23.5, SD 4.5) were included. All were veiled and covered heavily when outside the house for social and cultural reasons. Only eight (17%) had adequate varied diet with daily milk ingestion. All patients presented with progressive proximal muscle weakness lasting 6-24 months (mean 14) prior to our evaluation. The weakness was severe in six (13%) patients leading to wheel chair bound states. Associated musculoskeletal pain involving the back, hips, or lower limbs was common (66%). Osteomalcia was the referral diagnosis in only 11 patients and the remaining 36 (77%) patients were misdiagnosed. All patients had metabolic and radiological profiles suggestive of osteomalacia. Remarkable recovery was documented in all patients following oral cholecalciferol and calcium supplementation. CONCLUSIONS: Vitamin D deficiency is an important treatable cause of osteomalacic myopathy in Saudi Arabia. The diagnosis is frequently delayed or missed. Screening for Vitamin D deficiency in patients with acquired myopathy is needed to identify this treatable disorder.

Subacute sclerosing panencephalitis presenting with unilateral periodic myoclonic jerks.

BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a rare complication of measles virus infection. The disease is characterized by behavioural abnormalities, intellectual deterioration, motor weakness, and generalized myoclonic jerks progressing to coma and death in one to two years in 80% of the cases. The myoclonic jerks are associated with characteristic generalized slow periodic complexes on electroencephalography (EEG). The symptoms and signs of SSPE are frequently quite variable. The clinical course is equally variable and difficult to predict. The characteristic periodic myoclonus can rarely occur unilaterally particularly in the early stages of the disease. As well, the periodic EEG complexes have been reported unilaterally in up to 3% of cases. CASE REPORT: A 12-year-old boy, who was seen at a later stage with atypical manifestation of myoclonic body jerks confined entirely unilaterally, combined with contralateral periodic EEG complexes. One could assume clinically that the more diseased hemisphere was responsible for generating the jerks. However, brain magnetic resonance imaging revealed asymmetric hemispheric changes suggesting that the less neurologically damaged hemisphere is responsible for generating the unilateral myoclonic jerks. This has led to the interpretation that the more severely damaged hemisphere has lost the neuronal connectivity required to generate these periodic myoclonic jerks. CONCLUSIONS: Subacute sclerosing panencephalitis may have asymmetric hemispheric involvement, not only early, but also in the advanced stages of the disease, which can result in unilateral periodic myoclonic jerks.

Central fever due to hypothalamic lesion in a patient with tuberculous meningitis.

Abnormalities of body temperature are perhaps the most common features in many systemic pathologic processes. Such pathologic alterations are nearly always the result of extrinsic factors (for example, systemic pyrogens) which affect the hypothalamic thermoregulatory center by way of circulatory system. Much less common is alterations in temperature regulation resulting from intrinsic lesions of the thermoregulatory center in the hypothalamus. We report a patient with tuberculous meningitis who continued to have persistent fever despite the satisfactory treatment of her tuberculosis. A central thermoregulatory defect was documented and was attributed to a small structural lesion in the anterior hypothalamus.