Interferon alpha in subacute sclerosing panencephalitis: Case report and review of the literature

Figures & data

Table 1: Summary of epidemiological characteristics of described SSPE cases from published South African studies ¹

Reference	Year of data collection	Area	Total number of cases	SSPE incidence ^a	No of cases by race	Age
^9,10	1971–1974	Cape Province	15	1.2	9M,6B	3–13
^14	1955–1974	Southern Africa ^b	79	ND	36W,23M,20B	1–23
^14	1955–1975	Southern Africa ^b	96	M,15.9 ^c ,W,2.9 ^c	30W,30M,26B	1–23
^6	1955–1980	Southern Africa ^b	116	M: 2.6 ^c ^, W:1.47 ^c	ND	ND
^7	1984–1987	South Africa	44	Overall: 0.43,W:0.63,B:0.36	12W,32B	3–27
^8	1984–1990	South Africa	75	ND	16W,5M,53B	2–29
^10	1982–1987	KwaZulu Natal	18	ND	17B,1A ^d	4–14

^a Number of cases per 100 000 people per year. W: white; M: Mixed; B: Black; ND: not done

^b Includes Zimbabwe, Namibia and Malawi

^c Age Specific (0–24yrs) incidence, Cape Province only (1970–1976)

^d A: Asian However no cases have been documented regarding successful treatment of patients with SSPE from Africa. To our knowledge this is the first documented case using intraventricular interferon alpha in Africa

Figure 1: Patient was quadrispastic and bedbound.

Figure 2: EEG shows a slow background with periodic complexes coincident with patient’s jerks.

Table 2: Investigations on admission

Blood tests		Reference Ranges
Haemoglobin	13.3 g/dl	12–15 g/dl
Platelets	397 × 10^9 cells/l	186-450 × 10^9 cells/l
White blood cell count	5 × 10^9 cells/l	4–12 × 10^9 cells/l
Erthrocyte sedimentation rate	56 mm/hr	0–10 mm/hr
Urea and electrolytes	Normal
Calcium, magnesium and phosphate	Normal
Thyroid function test	Normal
Copper	Normal
Caeruloplasmin	Normal
Human immunodeficiency virus (X2)	Negative
Rapid Plasma Reagin test	Negative
Antinuclear factor	Negative
Measles IgG	Positive
Cerebral Spinal Fluid (CSF) tests
Protein	0.08 g/l	0.15–0.45 g/l
Globulins	Not increased
Glucose	5.3 mmol/l	2.2–3.9 mmol/l
PCR (HSV, CMV, HZ, enteroviruses)	Negative
Cytology	Negative
Measles-specific oligoclonal bands	Positive
Measles-specific ELISA antibodies	Positive
Imaging
CT brain	Normal
MRI	White matter hyperintensities
CXR	Normal

Figure 3: MRI brain at admission shows periventricular white matter hyperintensities.

Figure 4: EEG at 9 months post treatment (July 2007) showing significant improvement in the EEG with a normal background without periodic complexes.

Figure 5: Patient is well, able to walk and converse normally and has no myoclonic jerks.

Figure 6: Brain MRI 30 months after treatment (April 2008), showing progressive periventricular white matter hyperintensities.

Figure 7: Chest X-ray 30 months after treatment (April 2008), showing hilar infiltrates.

Table 3: Outcome of patients treated with intraventricular IFNα

Author	Reference	Place and date of Study	Number of Patients	Maximum Dosage	Duration of Follow Up	Outcome
Banu Anlar et al.	^24	Turkey(1986–1991)	22	I MU/week	56–108 months	50% Remission, 22% Stabilised, 28% Progressed
Masahito Miyazaki	^25	Japan(1985–2003)	1	3 MU/week	18 years	Remission for 8 years
Panitchi	^26	Japan(1986)	3 (Stage 2/3)	unknown	± 2 years	100% Remission
Steiner	^27	Israel(1989)	3	1 MU twice/week	± 2 years	100% remission
Kurata	^28	Japan	1 (Stage 2B)	3 MU/week	6 months	Improved
Horiquchi	^29	Japan	1	6 MU/week	4 years	Improved

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