Zilber et al. (1991) calculated the rate of CJD among Jews born in Egypt to be 3.5 per million [See: Libya > Zilber, et al. (1991)].

Kahana et al. (1974) was the first to describe the aggregation of CJD cases among Libyan Jews. They reported the occurrence of 29 cases with onset between 1963 and 1972. Incidence of the disease among Jewish immigrants from Libya was 31.3 per million.

In a country-wide survey of CJD in the Occupied Territories, Zilber et al. (1991) diagnosed 114 cases, among them 49 Libyan-born, with onset of disease during the years 1963-1987. After age adjustment, the mean annual incidence rate per million population was 43 among Libyan-born. Among Libyan Jews, there was no association between incidence rate of CJD and age at immigration, i.e., duration of exposure to a hypothetical infectious factor in Libya. The percentage of familial cases among Libyan Jews (41 to 47%) is one of the highest known. Kahana et al. (1991) reported that the clinical presentation and evolution of the disease were very similar in patients born in Libya and others without Libyan ancestry but tended to be more classic in the Libyan patients, with higher frequency of myoclonic jerks and periodic EEG and a progressive course of shorter duration.

Goldfarb et al. (1991) identified the E200K mutation in 45 of 55 CJD-affected families studied at the NIH laboratory. The families comprised a total of 87 patients and originated from seven different countries, including Libya.

Few years later, Meiner et al. (1997) reviewed familial Creutzfeldt-Jakob disease with particular reference to the E200K mutation, which is unusually frequent in Libyan Jews.

Two Omani patients with Creutzfeldt-Jakob disease (CJD) were described by Scrimgeour et al. (1996). The first patient was a 50-year old male who developed trembling movements of the left upper limb, which then progressed within three months into generalized myoclonic jerks with associated memory loss and disorientation. On admission, he was conscious but unresponsive, reacted to pain by withdrawal, had deterioration of higher cortical functions, behavioral abnormalities, and primitive reflexes were present. Other findings were extrapyramidal rigidity, cerebellar signs, and stimulus sensitive myoclonus. No abnormality was detected on hematological, biochemical and serological investigations. CSF examination revealed clear and acellular fluid with normal protein and glucose levels, and no virus was detected by either electron microscopy or tissue culture. Mild cerebral atrophy was evident on CT scan of the brain. EEG was supportive of the clinical diagnosis of CJD, as it revealed background suppression with generalized periodic triphasic sharp wave discharges at a frequency of 0.5 to 1 per second, associated with myoclonic jerks. The patient's condition continued to deteriorate and he died at home. The second case was that of a 75-year old male who developed dizziness, vertigo, ataxia with falls, forgetfulness and visual impairment for six months. His condition then deteriorated within three months into memory loss, disorientation, inability to recognize his relatives and friends, and he developed sporadic jerking movements of the left arm and leg, which then progressed into generalized myoclonic jerks involving the trunk and all limbs. He became inert, dysphasic and within four weeks, became mute and unresponsive to speech. Initial CSF examination in the rural hospital revealed clear and acellular fluid with protein levels of 54 mg%. The patient was referred to the authors' care, where he was found to be conscious but mute and unresponsive to speech, with marked memory loss, higher cortical dysfunction, behavioral abnormalities, and had primitive reflexes. Other neurological findings were visual and oculomotor dysfunction, vertigo and dizziness, marked extrapyramidal rigidity, cerebellar signs, stimulus sensitive myoclonus, lower motor neuron signs, and fasciculations. Similar to the first patient, all hematological, biochemical and serological investigations were normal, and results of a repeat lumbar puncture were not different from the initial one, except for an elevated protein level of 96 mg%. CT scan of the brain revealed advanced cerebral atrophy with dilated ventricles, and EEG was supportive of the clinical diagnosis of CJD, as it showed periodic generalized sharp wave discharges at a frequency of 1 Hz, predominant over the left mid-temporal regions. The patient's condition suddenly deteriorated and then he died. Autopsy was not possible in the two patients, but in the second patient a specimen of CSF was stored at -80 degrees Celsius in which two protein spots (130 and 131) were detected by two-dimensional gel electrophoresis and silver staining. As these spots were only seen in patients with CJD and herpes simplex encephalitis, it was a diagnostic confirmation of CJD in the second patient who had clinical features of the disease and no features of herpes infection. Scrimgeour et al. (1996) highlighted that accurate estimation of the CJD incidence in the Middle East was only possible in the presence of diagnostic CSF tests in order to diagnose more patients.

Zilber et al. (1991) calculated the rate of CJD among Jews born in Tunisia to be 2.3 per million [See: Libya > Zilber, et al. (1991)].

Goldfarb et al. (1991) identified the E200K mutation in 45 of 55 CJD-affected families studied at the NIH laboratory. The families contained a total of 87 patients and originated from seven different countries, including Tunisia.