TABLE 2.
Clinical course and treatment of patients with prion disease following the advent of akinetic mutism.
| Administration |
||||||||
|---|---|---|---|---|---|---|---|---|
| Case No. | Diagnosis | Observation period after induction of tube-feeding (months) | Coexistent disease (Therapy) | Complication (No. of times) | Cause of death | Nutrition (Calories (kcal.) / day) | Intravenous (No. of times; total duration) | Transluminal (No. of times; total duration) |
| Present series | ||||||||
| 1 | V180I genetic CJD (Definite) | 3 | Hypertension, Hyperlipidemia (Hypotensor, Hyperlipidemia medicine) | BC (2) | RF | Gastrostomy (800 – 1,000) | None | Ab (2; 14 days) |
| 2 | V180I genetic CJD (Probable) | 39 | Hypertension (Hypotensor) | BC (6), Fo (6), Ph (1) | Alive | Gastrostomy (600 – 1,000) | No CI , Ab (1; 5 days) | Ab (13; 127 days) |
| 3 | V180I genetic CJD (Probable) | 35 | None | BC (5), Ph (1) | Alive | Gastrostomy (1,000) | None | Ab (6; 65 days) |
| Our previous report | ||||||||
| A | V180I gCJD (Definite) | 21 | Pemphigoid (Steroid) | BC (6), GE (1), | RF | Nasal-tube (800) | CI (4; 42 days)(including CVC (1; 6 days); No HA), Ab (5; 20 days) | Ab (4; 26 days) |
| B | sCJD (Definite) | 1 | Bronchial asthma (Bronchodilator) | AP (1), BC (1), | RF | Nasal-tube (800) | CI (1; 18 days)(including CVC (1; 16 days); HA (1; 11 days)), Ab (1; 16 days) | Ab (1; 4 days) |
| C | sCJD (Definite) | 23.5 | None | AP (2), BC (15), HZ (1), UTI (1) | RF | Nasal-tube (800 – 1,000) | CI (2; 45 days)(including CVC (2; 35 days); HA (2; 29 days)), Ab (3; 29 days) | Ab (24; 94 days), Av (1; 7 days) |
| D | P102L GSS (Definite) | 17.5 | None | AP (3), BC (2), Cellulitis (2), PM (1) | RF with AP | Nasal-tube (600 – 800) | CI (3; 49 days)(including CVC (3; 60 days); HA (3; 35 days)), Ab (4; 51 days), Af (1; 11 days) | Ab (3; 23 days) |
| E | sCJD (Definite) | 7 | None | BC (6), UTI (1), | RF | Nasal-tube (1,000 – 1,400) | None | Ab (7; 55 days) |
| F | sCJD (Definite) | 22 | None | BC (9), GE (1) | RF | Nasal-tube (1,000) | None | Ab (10; 74 days) |
| G | sCJD (Definite) | 8 | None | AP (1), ARF (1), GE (7), UTI (1) | ARF with AP | Nasal-tube (800 – 1000) | CI (5; 124 days)(including CVC (2; 4 days); HA (2; 58 days)), Ab (3; 33 days) | Ab (3; 21 days) |
| H | sCJD (Probable) | 8 | None | BC (2) | — | Nasal-tube (1,000) | None | Ab (2; 14 days) |
| I | sCJD (Definite) | 12 | None | AP (1), BC (1) | RF with AP | Nasal-tube (1,000 kcal) | CI (1; 1 day), Ab (1; 1 day) | Ab (1; 7 days) |
| J | sCJD (Definite) | 3.5 | None | AP(1), BC (1), UTI (1), GE (2) | RF with AP | Nasal-tube (1,200) | CI (1; 5 days)(including CVC (1; 3 days); HA (1; 2 days)), Ab (1; 5 days) | Ab (3; 18 days) |
| K | sCJD (Probable) | 8 | Hypertension (Hypotensor) | GE (2) | — | Nasal-tube (1,000) | CI (1; 10 days), Ab (1; 10 days) | Ab (1; 3 days) |
| L | sCJD (Probable) | 2 | None | BC (2) | — | Nasal-tube (1,000) | CI(1; 14 days), Ab (1; 14 days) | Ab (1; 5 days) |
CVC, central venous catheter; Ab, antibiotic; Af, antifungal; Av, antiviral.
AP, aspiration pneumonia; ARF, acute renal failure; BC, bronchitis; Fo, folliculitis; GE, gastroenteritis; HZ, herpes zoster; Ph, phlegmon; PM, pulmonary mycosis; RF, respiratory failure; UTI, urinary tract infection.
CI, continuous infusions, HA, hyperalimentation.
No. of times, Number of times that the treatment was administered; Total duration, the total length of time for which all treatments of the same type were administered.
Complication, Complication for which treatment was needed.
*
Modified from our previous study.4